Genetic Mouse Models / FMR1

FMR1 fragile X mental retardation 1

Model Species
Mus musculus
Gene Aliases
FMRP; Fmr-1
Reports
36
Associated Human Genes
FMR1
Models / Rescue Models
59 / 21
Rescue Model
-
Model Summary

Fragile X Syndrome.

Reports related to FMR1 (36 Reports)
# Type Title Author, Year
1 Additional Deletion of FMR1 in Purkinje cells enhances parallel fiber LTD, enlarges spines, and attenuates cerebellar eyelid conditioning in Fragile X syndrome. Koekkoek SK , et al. (2005)
2 Additional Expression profiling suggests underexpression of the GABA(A) receptor subunit delta in the fragile X knockout mouse model. Gantois I , et al. (2005)
3 Additional The generation of a conditional Fmr1 knock out mouse model to study Fmrp function in vivo. Mientjes EJ , et al. (2005)
4 Additional Major defects in neocortical GABAergic inhibitory circuits in mice lacking the fragile X mental retardation protein. Selby L , et al. (2007)
5 Additional Fragile X mental retardation protein regulates the levels of scaffold proteins and glutamate receptors in postsynaptic densities. Schtt J , et al. (2009)
6 Additional Fragile X mental retardation protein is required for chemically-induced long-term potentiation of the hippocampus in adult mice. Shang Y , et al. (2009)
7 Additional Fmr1 knockout mice show reduced anxiety and alterations in neurogenesis that are specific to the ventral dentate gyrus. Eadie BD , et al. (2009)
8 Additional Lithium ameliorates altered glycogen synthase kinase-3 and behavior in a mouse model of fragile X syndrome. Yuskaitis CJ , et al. (2009)
9 Additional Altered neuroligin expression is involved in social deficits in a mouse model of the fragile X syndrome. Dahlhaus R and El-Husseini A (2009)
10 Additional A mouse model of the human Fragile X syndrome I304N mutation. Zang JB , et al. (2009)
11 Additional Dysregulation of mTOR signaling in fragile X syndrome. Sharma A , et al. (2010)
12 Additional GSK3 influences social preference and anxiety-related behaviors during social interaction in a mouse model of fragile X syndrome and autism. Mines MA , et al. (2010)
13 Additional A mouse model of the fragile X premutation: effects on behavior, dendrite morphology, and regional rates of cerebral protein synthesis. Qin M , et al. (2011)
14 Additional Hyperconnectivity and slow synapses during early development of medial prefrontal cortex in a mouse model for mental retardation and autism. Testa-Silva G , et al. (2011)
15 Additional Reversal of fragile X phenotypes by manipulation of APP/A levels in Fmr1KO mice. Westmark CJ , et al. (2011)
16 Additional The GABA(A) receptor agonist THIP ameliorates specific behavioral deficits in the mouse model of fragile X syndrome. Olmos-Serrano JL , et al. (2011)
17 Additional Minocycline treatment reverses ultrasonic vocalization production deficit in a mouse model of Fragile X Syndrome. Rotschafer SE , et al. (2012)
18 Additional Chronic pharmacological mGlu5 inhibition corrects fragile X in adult mice. Michalon A , et al. (2012)
19 Additional Activation of 5-HT7 serotonin receptors reverses metabotropic glutamate receptor-mediated synaptic plasticity in wild-type and Fmr1 knockout mice, ... Costa L , et al. (2012)
20 Additional Genetic removal of p70 S6 kinase 1 corrects molecular, synaptic, and behavioral phenotypes in fragile X syndrome mice. Bhattacharya A , et al. (2012)
21 Additional Reorganization of circuits underlying cerebellar modulation of prefrontal cortical dopamine in mouse models of autism spectrum disorder. Rogers TD , et al. (2013)
22 Additional Rescue of fragile X syndrome phenotypes in Fmr1 KO mice by the small-molecule PAK inhibitor FRAX486. Dolan BM , et al. (2013)
23 Additional Long-lasting effects of minocycline on behavior in young but not adult Fragile X mice. Dansie LE , et al. (2013)
24 Additional Pharmacological rescue of Ras signaling, GluA1-dependent synaptic plasticity, and learning deficits in a fragile X model. Lim CS , et al. (2014)
25 Additional Oxytocin-mediated GABA inhibition during delivery attenuates autism pathogenesis in rodent offspring. Tyzio R , et al. (2014)
26 Additional Hyperactivity and lack of social discrimination in the adolescent Fmr1 knockout mouse. Srensen EM , et al. (2015)
27 Additional Convergence of Hippocampal Pathophysiology in Syngap and Fmr1-/y Mice. Barnes SA , et al. (2015)
28 Additional Peripheral Mechanosensory Neuron Dysfunction Underlies Tactile and Behavioral Deficits in Mouse Models of ASDs. Orefice LL , et al. (2016)
29 Additional Excess Translation of Epigenetic Regulators Contributes to Fragile X Syndrome and Is Alleviated by Brd4 Inhibition. Korb E , et al. (2017)
30 Additional Adult Fmr1 knockout mice present with deficiencies in hippocampal interleukin-6 and tumor necrosis factor- expression. Hodges SL , et al. (2017)
31 Additional Chronic minocycline treatment improves hippocampal neuronal structure, NMDA receptor function, and memory processing in Fmr1 knockout mice. Yau SY , et al. (2018)
32 Additional Striatal Inhibition of MeCP2 or TSC1 Produces Sociability Deficits and Repetitive Behaviors. Lee Y , et al. (2019)
33 Additional Increased Excitation-Inhibition Ratio Stabilizes Synapse and Circuit Excitability in Four Autism Mouse Models. Antoine MW , et al. (2019)
34 Additional Targeting Peripheral Somatosensory Neurons to Improve Tactile-Related Phenotypes in ASD Models. Orefice LL , et al. (2019)
35 Primary Fmr1 knockout mice: a model to study fragile X mental retardation. The Dutch-Belgian Fragile X Consortium. no authors (1994)
36 Primary Abnormal dendritic spines in fragile X knockout mice: maturation and pruning deficits. Comery TA , et al. (1997)
Show all Construct Details Show all Phenotypic Profiles

M_FMR1_10_KO_HM

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

Purkinje cell specific knock out of FMR1, using L7-cre mice.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: mixed

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Koekkoek SK , et al. (2005)
Circadian sleep/wake cycle - Not reported - - Koekkoek SK , et al. (2005)
Motor phenotype Spontaneous movement Increased Home cage behavior Unreported Koekkoek SK , et al. (2005)
Neuroanatomy / ultrastructure / cytoarchitecture Brain size No change Measurement of tissue weight 2 months Koekkoek SK , et al. (2005)
Neurophysiology - Not reported - - Koekkoek SK , et al. (2005)
Repetitive behavior - Not reported - - Koekkoek SK , et al. (2005)
Seizure - Not reported - - Koekkoek SK , et al. (2005)
Sensory - Not reported - - Koekkoek SK , et al. (2005)
Social behavior Social interaction No change Reciprocal social interaction test Unreported Koekkoek SK , et al. (2005)
Social behavior Social interaction Increased Light-dark exploration test Unreported Koekkoek SK , et al. (2005)
Communications - Not reported - - Koekkoek SK , et al. (2005)
Developmental profile Size/growth Increased Measurement of tissue weight 5-7 weeks, 2.5 months; 3.5 months; 5.5 months Koekkoek SK , et al. (2005)
Developmental profile Reproductive system development No change General observations Unreported Koekkoek SK , et al. (2005)
Emotion - Not reported - - Koekkoek SK , et al. (2005)
Immune response - Not reported - - Koekkoek SK , et al. (2005)
Learning & memory Spatial learning No change Morris water maze test Unreported Koekkoek SK , et al. (2005)
Learning & memory Spatial learning Decreased Morris water maze test Unreported Koekkoek SK , et al. (2005)
Learning & memory Cued or contextual fear conditioning No change Fear conditioning test Unreported Koekkoek SK , et al. (2005)
Maternal behavior - Not reported - - Koekkoek SK , et al. (2005)
Molecular profile - Not reported - - Koekkoek SK , et al. (2005)

M_FMR1_11_KO_HM

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

FMR1 KO mice with a mixed FVB and 129P2 background(information not available for pde6b+.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: FVB.129P2

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Sharma A , et al. (2010)
Circadian sleep/wake cycle - Not reported - - Sharma A , et al. (2010)
Motor phenotype - Not reported - - Sharma A , et al. (2010)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Sharma A , et al. (2010)
Neurophysiology - Not reported - - Sharma A , et al. (2010)
Repetitive behavior - Not reported - - Sharma A , et al. (2010)
Seizure - Not reported - - Sharma A , et al. (2010)
Sensory - Not reported - - Sharma A , et al. (2010)
Social behavior - Not reported - - Sharma A , et al. (2010)
Communications - Not reported - - Sharma A , et al. (2010)
Developmental profile - Not reported - - Sharma A , et al. (2010)
Emotion - Not reported - - Sharma A , et al. (2010)
Immune response - Not reported - - Sharma A , et al. (2010)
Learning & memory - Not reported - - Sharma A , et al. (2010)
Maternal behavior - Not reported - - Sharma A , et al. (2010)
Molecular profile Regulation of gene expression Increased Western blot 4-6 weeks Sharma A , et al. (2010)
Molecular profile Protein phosphorylation No change Western blot 4-6 weeks Sharma A , et al. (2010)
Molecular profile Protein phosphorylation Increased Western blot 4-6 weeks, 6-8 months Sharma A , et al. (2010)
Molecular profile Protein expression level evidence No change Western blot 4-6 weeks; 6-8 months Sharma A , et al. (2010)
Molecular profile Enzyme activity No change Western blot 4-6 weeks Sharma A , et al. (2010)
Molecular profile Enzyme activity Increased Western blot 4-6 weeks Sharma A , et al. (2010)

M_FMR1_12_KO_HM

Model Type: Genetic
Model Genotype: Homozygous; hemizygous

Mutation:

FMR1 knockout mice on the FVB/NJ background. (Construct details not available).

Allele Type: Knockout
Strain of Origin: Not Specified
Genetic Background: FVB/NJ

Es cell line: Not Specified
Model Source: Lugo Lab, Baylor University (PMID 28915148)
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Hodges SL , et al. (2017)
Circadian sleep/wake cycle - Not reported - - Hodges SL , et al. (2017)
Motor phenotype - Not reported - - Hodges SL , et al. (2017)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Hodges SL , et al. (2017)
Neurophysiology - Not reported - - Hodges SL , et al. (2017)
Repetitive behavior - Not reported - - Hodges SL , et al. (2017)
Seizure - Not reported - - Hodges SL , et al. (2017)
Sensory - Not reported - - Hodges SL , et al. (2017)
Social behavior - Not reported - - Hodges SL , et al. (2017)
Communications - Not reported - - Hodges SL , et al. (2017)
Developmental profile - Not reported - - Hodges SL , et al. (2017)
Emotion - Not reported - - Hodges SL , et al. (2017)
Immune response Cytokine levels: pro-inflammatory No change Quantitative pcr (qrt-pcr) Adult Hodges SL , et al. (2017)
Immune response Cytokine levels: pro-inflammatory Decreased Quantitative pcr (qrt-pcr) Adult Hodges SL , et al. (2017)
Immune response Cytokine levels: chemokine No change Quantitative pcr (qrt-pcr) Adult Hodges SL , et al. (2017)
Immune response Cytokine levels: anti-inflammatory No change Quantitative pcr (qrt-pcr) Adult Hodges SL , et al. (2017)
Learning & memory - Not reported - - Hodges SL , et al. (2017)
Maternal behavior - Not reported - - Hodges SL , et al. (2017)
Molecular profile - Not reported - - Hodges SL , et al. (2017)

M_FMR1_13_KD

Model Type: Genetic
Model Genotype: Wildtype

Mutation:

Seven-week-old wildtype C57BL/6 male mice were injected with Fmr1-siRNA (1358762, NM_008031.2), bilaterally in the dorsal striatum using a stereotaxic apparatus, resulting in a reduction of Fmr1 transcript levels to 0.4 fold compared with control siRNA injected mice.

Allele Type: Knockdown
Strain of Origin: C57BL/6
Genetic Background: C57BL/6

Es cell line: NA
Model Source: Daehan BioLink (Eumsung, Chungbuk, Republic of Korea)
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Lee Y , et al. (2019)
Circadian sleep/wake cycle - Not reported - - Lee Y , et al. (2019)
Motor phenotype - Not reported - - Lee Y , et al. (2019)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lee Y , et al. (2019)
Neurophysiology - Not reported - - Lee Y , et al. (2019)
Repetitive behavior Self grooming: perseveration No change Grooming behavior assessments Adult Lee Y , et al. (2019)
Repetitive behavior Repetitive digging No change Home cage behavior Adult Lee Y , et al. (2019)
Seizure - Not reported - - Lee Y , et al. (2019)
Sensory - Not reported - - Lee Y , et al. (2019)
Social behavior Social memory Decreased U-field assay Adult Lee Y , et al. (2019)
Communications - Not reported - - Lee Y , et al. (2019)
Developmental profile - Not reported - - Lee Y , et al. (2019)
Emotion - Not reported - - Lee Y , et al. (2019)
Immune response - Not reported - - Lee Y , et al. (2019)
Learning & memory Object recognition memory Decreased Object preference test Adult Lee Y , et al. (2019)
Maternal behavior - Not reported - - Lee Y , et al. (2019)
Molecular profile Targeted expression Decreased Quantitative pcr (qrt-pcr) Adult Lee Y , et al. (2019)

M_FMR1_14_KO_HE

Model Type: Genetic
Model Genotype: Homozygous/Hemizygous

Mutation:

FMR1 mutant gene was generated by construction a targeting vector using homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin (neo). FMR1 homozygous knockout mice (FMR1^tm1Cgr) were bred with NeuroD1 bacTRAP (ND1-BT) heterozygous mice (PMID 28823556) to generate FMR1 and ND-BT1 dual heterozygous female mice that were bred with FMR1-/y male mice to generate FMR1 null and ND1-BT heterozygous (FMR1-/-;ND1-BT /-) that were used for experimentations; M_FMR1_1_KO_HM

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6J*129/SvEv*CD1

Es cell line: -
Model Source: Not specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Sensory Startle response: acoustic stimulus No change Acoustic startle reflex test 6-8 weeks Orefice LL , et al. (2019)
Sensory Sensorimotor gating: tactile cue Increased Response to air puff 6-8 weeks Orefice LL , et al. (2019)
Sensory Sensorimotor gating: tactile cue Increased Prepulse inhibition 6-8 weeks Orefice LL , et al. (2019)

M_FMR1_14_KO_HE_ISOGUVACINE

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous/Hemizygous

Mutation:

Isoguvacine is a potent and selective agonist for the GABAAR that does not cross the blood brain barrier and so is a peripherally restricted GABAAR agonist. Isoguvacine, 2 mg/kg, i.p., was administered beginning at a young age (P1-P42) to test if it ameliorated tactile hypersensitivity in ASD models with fewer adverse effects than brain-penetrating benzodiazepines such as midazolam, including sedation and cognitive impairment, which are attributed to a CNS site of drug action.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6J*129/SvEv*CD1

Es cell line: -
Model Source: Not specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Sensory Startle response: acoustic stimulus No adverse effect Acoustic startle reflex test 6 weeks Orefice LL , et al. (2019)
Sensory Sensorimotor gating: tactile cue Restored Response to air puff 6 weeks Orefice LL , et al. (2019)
Sensory Sensorimotor gating: tactile cue Restored Prepulse inhibition 6 weeks Orefice LL , et al. (2019)

M_FMR1_1_KO_HM

Model Type: Genetic
Model Genotype: Homozygous; hemizygous

Mutation:

FMR1 mutant gene was generated by construction a targeting vector using homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin (neo). FMR1 homozygous knockout mice (FMR1^tm1Cgr) were bred with NeuroD1 bacTRAP (ND1-BT) heterozygous mice (PMID 28823556) to generate FMR1 and ND-BT1 dual heterozygous female mice that were bred with FMR1-/y male mice to generate FMR1 null and ND1-BT heterozygous (FMR1-/-;ND1-BT+/-) that were used for experimentations.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6J

Es cell line: E14
Model Source: The Jackson Laboratory
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Comery TA , et al. (1997)
Physiological parameters - Not reported - - Yau SY , et al. (2018)
Physiological parameters - Not reported - - Korb E , et al. (2017)
Physiological parameters - Not reported - - Srensen EM , et al. (2015)
Physiological parameters - Not reported - - Lim CS , et al. (2014)
Physiological parameters - Not reported - - Rogers TD , et al. (2013)
Physiological parameters - Not reported - - Michalon A , et al. (2012)
Physiological parameters - Not reported - - Rotschafer SE , et al. (2012)
Physiological parameters - Not reported - - Westmark CJ , et al. (2011)
Physiological parameters - Not reported - - Testa-Silva G , et al. (2011)
Physiological parameters - Not reported - - Mines MA , et al. (2010)
Physiological parameters - Not reported - - Dahlhaus R and El-Husseini A (2009)
Physiological parameters Hormone levels No change Elisa Unreported Eadie BD , et al. (2009)
Physiological parameters Hormone levels Decreased Elisa Unreported Eadie BD , et al. (2009)
Physiological parameters - Not reported - - Eadie BD , et al. (2009)
Physiological parameters - Not reported - - Schtt J , et al. (2009)
Physiological parameters - Not reported - - Gantois I , et al. (2005)
Circadian sleep/wake cycle - Not Reported - - -
Circadian sleep/wake cycle - Not reported - - Comery TA , et al. (1997)
Circadian sleep/wake cycle - Not reported - - Yau SY , et al. (2018)
Circadian sleep/wake cycle - Not reported - - Korb E , et al. (2017)
Circadian sleep/wake cycle - Not reported - - Srensen EM , et al. (2015)
Circadian sleep/wake cycle - Not reported - - Lim CS , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Rogers TD , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Michalon A , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Rotschafer SE , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Westmark CJ , et al. (2011)
Circadian sleep/wake cycle - Not reported - - Testa-Silva G , et al. (2011)
Circadian sleep/wake cycle - Not reported - - Mines MA , et al. (2010)
Circadian sleep/wake cycle - Not reported - - Dahlhaus R and El-Husseini A (2009)
Circadian sleep/wake cycle - Not reported - - Eadie BD , et al. (2009)
Circadian sleep/wake cycle - Not reported - - Schtt J , et al. (2009)
Circadian sleep/wake cycle - Not reported - - Gantois I , et al. (2005)
Motor phenotype - Not Reported - - -
Motor phenotype - Not reported - - Comery TA , et al. (1997)
Motor phenotype - Not reported - - Yau SY , et al. (2018)
Motor phenotype General locomotor activity: ambulatory activity No change Open field test 5 weeks Korb E , et al. (2017)
Motor phenotype General locomotor activity: ambulatory activity Increased Open field test 4-8 weeks Korb E , et al. (2017)
Motor phenotype General locomotor activity: ambulatory activity Increased Novel object recognition test 4-8 weeks Korb E , et al. (2017)
Motor phenotype Hyperactivity Increased Three-chamber social approach test 7-10 weeks Srensen EM , et al. (2015)
Motor phenotype Hyperactivity Increased Open field test 7-10 weeks Srensen EM , et al. (2015)
Motor phenotype - Not reported - - Lim CS , et al. (2014)
Motor phenotype - Not reported - - Rogers TD , et al. (2013)
Motor phenotype General locomotor activity Increased Open field test 2-5 months Michalon A , et al. (2012)
Motor phenotype - Not reported - - Rotschafer SE , et al. (2012)
Motor phenotype General locomotor activity No change Open field test Unreported Westmark CJ , et al. (2011)
Motor phenotype - Not reported - - Testa-Silva G , et al. (2011)
Motor phenotype - Not reported - - Mines MA , et al. (2010)
Motor phenotype Swimming ability Increased Swim test 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Motor phenotype General locomotor activity Increased Open field test 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Motor phenotype General locomotor activity No change Open field test Unreported Eadie BD , et al. (2009)
Motor phenotype - Not reported - - Schtt J , et al. (2009)
Motor phenotype - Not reported - - Gantois I , et al. (2005)
Neuroanatomy / Ultrastructure / Cytoarchitecture - Not Reported - - -
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Increased Immunohistochemistry 8 weeks Comery TA , et al. (1997)
Neuroanatomy / ultrastructure / cytoarchitecture Neuroreceptor levels: glutamate receptors: nmda receptors Decreased Western blot Adult Yau SY , et al. (2018)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: dendritic tree complexity Decreased Sholl analysis Adult Yau SY , et al. (2018)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: dendritic length Decreased Sholl analysis Adult Yau SY , et al. (2018)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Increased Immunohistochemistry 4-8 weeks Korb E , et al. (2017)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Srensen EM , et al. (2015)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lim CS , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Rogers TD , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Increased Golgi-cox staining Unreported Michalon A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Rotschafer SE , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Westmark CJ , et al. (2011)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Testa-Silva G , et al. (2011)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Mines MA , et al. (2010)
Neuroanatomy / ultrastructure / cytoarchitecture Synapse density Increased Immunohistochemistry 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Neuroanatomy / ultrastructure / cytoarchitecture Synapse density Decreased Immunohistochemistry 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Neuroanatomy / ultrastructure / cytoarchitecture Brain development No change Immunohistochemistry Unreported Eadie BD , et al. (2009)
Neuroanatomy / ultrastructure / cytoarchitecture Brain development Decreased Immunohistochemistry Unreported Eadie BD , et al. (2009)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Schtt J , et al. (2009)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Gantois I , et al. (2005)
Neurophysiology Neuronal activation Increased Immunohistochemistry Adult, 2-5 months, 9-12 months -
Neurophysiology - Not reported - - Comery TA , et al. (1997)
Neurophysiology Synaptic neuroreceptor ratio (nmdar/ampar) dependent transmission Decreased Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology Miniature post synaptic current amplitude: excitatory No change Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology Miniature post synaptic current amplitude: excitatory Decreased Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology Epsp-spike relationship No change Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology Epsp-spike relationship Decreased Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology - Not reported - - Korb E , et al. (2017)
Neurophysiology Neurotransmitter release: catecholamines Increased High-performance liquid chromatography (hplc) 7-10 weeks Srensen EM , et al. (2015)
Neurophysiology - Not reported - - Lim CS , et al. (2014)
Neurophysiology Neurotransmitter release: catecholamines No change In vivo amperometry Unreported Rogers TD , et al. (2013)
Neurophysiology Neurotransmitter release: catecholamines Decreased In vivo amperometry Unreported Rogers TD , et al. (2013)
Neurophysiology Neurotransmitter release: catecholamines Decreased In vivo amperometry Unreported Rogers TD , et al. (2013)
Neurophysiology Synaptic plasticity: ltd Increased Field potential recordings 3-4 weeks Michalon A , et al. (2012)
Neurophysiology Synaptic plasticity Increased Field potential recordings 2 months Michalon A , et al. (2012)
Neurophysiology - Not reported - - Rotschafer SE , et al. (2012)
Neurophysiology Synaptic plasticity Increased Field potential recordings 3 months Westmark CJ , et al. (2011)
Neurophysiology Presynaptic function: paired-pulse facilitation No change Paired-pulse ratio Unreported Westmark CJ , et al. (2011)
Neurophysiology Synaptic transmission No change Whole-cell patch clamp 4-5 weeks Testa-Silva G , et al. (2011)
Neurophysiology Synaptic transmission Increased Whole-cell patch clamp Unreported Testa-Silva G , et al. (2011)
Neurophysiology Synaptic plasticity: ltd No change Whole-cell patch clamp 4-5 weeks Testa-Silva G , et al. (2011)
Neurophysiology Synaptic plasticity: ltd Decreased Whole-cell patch clamp Unreported Testa-Silva G , et al. (2011)
Neurophysiology - Not reported - - Mines MA , et al. (2010)
Neurophysiology - Not reported - - Dahlhaus R and El-Husseini A (2009)
Neurophysiology - Not reported - - Eadie BD , et al. (2009)
Neurophysiology - Not reported - - Schtt J , et al. (2009)
Neurophysiology - Not reported - - Gantois I , et al. (2005)
Repetitive behavior - Not Reported - - -
Repetitive behavior - Not reported - - Comery TA , et al. (1997)
Repetitive behavior - Not reported - - Yau SY , et al. (2018)
Repetitive behavior Repetitive digging Increased Marble-burying test 4.3 weeks, 6-8 weeks Korb E , et al. (2017)
Repetitive behavior Repetitive digging No change Marble-burying test 4-6 weeks Srensen EM , et al. (2015)
Repetitive behavior - Not reported - - Lim CS , et al. (2014)
Repetitive behavior - Not reported - - Rogers TD , et al. (2013)
Repetitive behavior - Not reported - - Michalon A , et al. (2012)
Repetitive behavior - Not reported - - Rotschafer SE , et al. (2012)
Repetitive behavior Repetitive digging Decreased Marble-burying test 8-10 weeks Westmark CJ , et al. (2011)
Repetitive behavior - Not reported - - Testa-Silva G , et al. (2011)
Repetitive behavior Self grooming: social context No change Three-chamber social approach test 3 months Mines MA , et al. (2010)
Repetitive behavior - Not reported - - Mines MA , et al. (2010)
Repetitive behavior - Not reported - - Dahlhaus R and El-Husseini A (2009)
Repetitive behavior - Not reported - - Eadie BD , et al. (2009)
Repetitive behavior - Not reported - - Schtt J , et al. (2009)
Repetitive behavior - Not reported - - Gantois I , et al. (2005)
Seizure - Not Reported - - -
Seizure - Not reported - - Comery TA , et al. (1997)
Seizure - Not reported - - Yau SY , et al. (2018)
Seizure - Not reported - - Korb E , et al. (2017)
Seizure - Not reported - - Srensen EM , et al. (2015)
Seizure - Not reported - - Lim CS , et al. (2014)
Seizure - Not reported - - Rogers TD , et al. (2013)
Seizure Seizures Increased Acoustic startle reflex test Unreported Michalon A , et al. (2012)
Seizure - Not reported - - Rotschafer SE , et al. (2012)
Seizure Seizures Increased Audiogenic seizure test P21 Westmark CJ , et al. (2011)
Seizure - Not reported - - Testa-Silva G , et al. (2011)
Seizure - Not reported - - Mines MA , et al. (2010)
Seizure - Not reported - - Dahlhaus R and El-Husseini A (2009)
Seizure - Not reported - - Eadie BD , et al. (2009)
Seizure - Not reported - - Schtt J , et al. (2009)
Seizure - Not reported - - Gantois I , et al. (2005)
Sensory - Not Reported - - -
Sensory - Not reported - - Comery TA , et al. (1997)
Sensory - Not reported - - Yau SY , et al. (2018)
Sensory - Not reported - - Korb E , et al. (2017)
Sensory - Not reported - - Srensen EM , et al. (2015)
Sensory - Not reported - - Lim CS , et al. (2014)
Sensory - Not reported - - Rogers TD , et al. (2013)
Sensory Startle response: acoustic stimulus Increased Acoustic startle reflex test Unreported Michalon A , et al. (2012)
Sensory - Not reported - - Rotschafer SE , et al. (2012)
Sensory - Not reported - - Westmark CJ , et al. (2011)
Sensory - Not reported - - Testa-Silva G , et al. (2011)
Sensory - Not reported - - Mines MA , et al. (2010)
Sensory - Not reported - - Dahlhaus R and El-Husseini A (2009)
Sensory - Not reported - - Eadie BD , et al. (2009)
Sensory - Not reported - - Schtt J , et al. (2009)
Sensory - Not reported - - Gantois I , et al. (2005)
Social behavior Social approach Decreased Three-chamber social approach test Adult -
Social behavior - Not reported - - Comery TA , et al. (1997)
Social behavior - Not reported - - Yau SY , et al. (2018)
Social behavior Social approach Increased Three-chamber social approach test 4.3 weeks, 6-8 weeks Korb E , et al. (2017)
Social behavior Social memory Decreased Three-chamber social approach test 7-10 weeks Srensen EM , et al. (2015)
Social behavior Social approach No change Three-chamber social approach test 7-10 weeks Srensen EM , et al. (2015)
Social behavior - Not reported - - Lim CS , et al. (2014)
Social behavior - Not reported - - Rogers TD , et al. (2013)
Social behavior - Not reported - - Michalon A , et al. (2012)
Social behavior Mating behavior No change Reciprocal social interaction test Unreported Rotschafer SE , et al. (2012)
Social behavior - Not reported - - Westmark CJ , et al. (2011)
Social behavior - Not reported - - Testa-Silva G , et al. (2011)
Social behavior Social memory Decreased Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social interaction No change Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social interaction No change Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social interaction No change Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social interaction Increased Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social interaction Increased Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social interaction Decreased Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social approach No change Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social approach No change Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social approach Increased Three-chamber social approach test 3 months Mines MA , et al. (2010)
Social behavior Social interaction Decreased Reciprocal social interaction test 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Social behavior Social interaction Decreased Reciprocal social interaction test 2-6 months Dahlhaus R and El-Husseini A (2009)
Social behavior Social interaction Decreased Resident-intruder test 2-6 months Dahlhaus R and El-Husseini A (2009)
Social behavior Aggression No change Resident-intruder test 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Social behavior - Not reported - - Eadie BD , et al. (2009)
Social behavior - Not reported - - Schtt J , et al. (2009)
Social behavior - Not reported - - Gantois I , et al. (2005)
Communications - Not Reported - - -
Communications - Not reported - - Comery TA , et al. (1997)
Communications - Not reported - - Yau SY , et al. (2018)
Communications - Not reported - - Korb E , et al. (2017)
Communications - Not reported - - Srensen EM , et al. (2015)
Communications Ultrasonic vocalization No change Monitoring ultrasonic vocalizations Unreported Lim CS , et al. (2014)
Communications - Not reported - - Rogers TD , et al. (2013)
Communications - Not reported - - Michalon A , et al. (2012)
Communications Ultrasonic vocalization Decreased Monitoring ultrasonic vocalizations Unreported Rotschafer SE , et al. (2012)
Communications - Not reported - - Westmark CJ , et al. (2011)
Communications - Not reported - - Testa-Silva G , et al. (2011)
Communications - Not reported - - Mines MA , et al. (2010)
Communications - Not reported - - Dahlhaus R and El-Husseini A (2009)
Communications - Not reported - - Eadie BD , et al. (2009)
Communications - Not reported - - Schtt J , et al. (2009)
Communications - Not reported - - Gantois I , et al. (2005)
Developmental profile - Not Reported - - -
Developmental profile - Not reported - - Comery TA , et al. (1997)
Developmental profile - Not reported - - Yau SY , et al. (2018)
Developmental profile - Not reported - - Korb E , et al. (2017)
Developmental profile - Not reported - - Srensen EM , et al. (2015)
Developmental profile - Not reported - - Lim CS , et al. (2014)
Developmental profile - Not reported - - Rogers TD , et al. (2013)
Developmental profile Tissue weight Increased General observations 5-17 weeks Michalon A , et al. (2012)
Developmental profile - Not reported - - Rotschafer SE , et al. (2012)
Developmental profile - Not reported - - Westmark CJ , et al. (2011)
Developmental profile - Not reported - - Testa-Silva G , et al. (2011)
Developmental profile - Not reported - - Mines MA , et al. (2010)
Developmental profile Size/growth Increased General observations 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Developmental profile Mortality/lethality No change General observations 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Developmental profile - Not reported - - Eadie BD , et al. (2009)
Developmental profile - Not reported - - Schtt J , et al. (2009)
Developmental profile - Not reported - - Gantois I , et al. (2005)
Emotion - Not Reported - - -
Emotion - Not reported - - Comery TA , et al. (1997)
Emotion Exploratory activity: habituation No change Object-place recognition test Adult Yau SY , et al. (2018)
Emotion Depression No change Forced swim test Adult Yau SY , et al. (2018)
Emotion Depression No change Tail suspension test Adult Yau SY , et al. (2018)
Emotion Anxiety No change Open field test Adult Yau SY , et al. (2018)
Emotion Anxiety No change Open field test 6-8 weeks Korb E , et al. (2017)
Emotion Anxiety Increased Open field test 5 weeks Korb E , et al. (2017)
Emotion Anxiety Increased Open field test 7-10 weeks Srensen EM , et al. (2015)
Emotion Anxiety Decreased Elevated plus maze test P29 Lim CS , et al. (2014)
Emotion Anxiety Decreased Light-dark exploration test P29 Lim CS , et al. (2014)
Emotion - Not reported - - Rogers TD , et al. (2013)
Emotion - Not reported - - Michalon A , et al. (2012)
Emotion - Not reported - - Rotschafer SE , et al. (2012)
Emotion Anxiety Decreased Open field test Unreported Westmark CJ , et al. (2011)
Emotion - Not reported - - Testa-Silva G , et al. (2011)
Emotion Anxiety Increased Three-chamber social approach test 3 months Mines MA , et al. (2010)
Emotion Anxiety Decreased Three-chamber social approach test 3 months Mines MA , et al. (2010)
Emotion - Not reported - - Dahlhaus R and El-Husseini A (2009)
Emotion Anxiety Decreased Elevated plus maze test 2-4 months Eadie BD , et al. (2009)
Emotion Anxiety Decreased Open field test 2-4 months Eadie BD , et al. (2009)
Emotion - Not reported - - Schtt J , et al. (2009)
Emotion - Not reported - - Gantois I , et al. (2005)
Homeostasis - Not Reported - - -
Immune response - Not Reported - - -
Immune response - Not reported - - Comery TA , et al. (1997)
Immune response - Not reported - - Yau SY , et al. (2018)
Immune response - Not reported - - Korb E , et al. (2017)
Immune response - Not reported - - Srensen EM , et al. (2015)
Immune response - Not reported - - Lim CS , et al. (2014)
Immune response - Not reported - - Rogers TD , et al. (2013)
Immune response - Not reported - - Michalon A , et al. (2012)
Immune response - Not reported - - Rotschafer SE , et al. (2012)
Immune response - Not reported - - Westmark CJ , et al. (2011)
Immune response - Not reported - - Testa-Silva G , et al. (2011)
Immune response - Not reported - - Mines MA , et al. (2010)
Immune response - Not reported - - Dahlhaus R and El-Husseini A (2009)
Immune response - Not reported - - Eadie BD , et al. (2009)
Immune response - Not reported - - Schtt J , et al. (2009)
Immune response - Not reported - - Gantois I , et al. (2005)
Learning & memory - Not Reported - - -
Learning & memory - Not reported - - Comery TA , et al. (1997)
Learning & memory Spatial reference memory Decreased Object-place recognition test Adult Yau SY , et al. (2018)
Learning & memory Procedural learning No change Novel object recognition test Adult Yau SY , et al. (2018)
Learning & memory Object recognition memory Decreased Novel object recognition test Adult Yau SY , et al. (2018)
Learning & memory Object recognition memory Decreased Novel object recognition test 4-8 weeks Korb E , et al. (2017)
Learning & memory - Not reported - - Srensen EM , et al. (2015)
Learning & memory Reward reinforced choice behavior Decreased Y-maze test P29 Lim CS , et al. (2014)
Learning & memory Eye blink conditioning Decreased Eyeblink conditioning Unreported Lim CS , et al. (2014)
Learning & memory Cued or contextual fear conditioning Decreased Fear conditioning test P29 Lim CS , et al. (2014)
Learning & memory Cued or contextual fear conditioning Decreased Fear conditioning test P29 Lim CS , et al. (2014)
Learning & memory - Not reported - - Rogers TD , et al. (2013)
Learning & memory Cued or contextual fear conditioning: memory of cue Decreased Passive avoidance test Unreported Michalon A , et al. (2012)
Learning & memory - Not reported - - Rotschafer SE , et al. (2012)
Learning & memory - Not reported - - Westmark CJ , et al. (2011)
Learning & memory - Not reported - - Testa-Silva G , et al. (2011)
Learning & memory - Not reported - - Mines MA , et al. (2010)
Learning & memory Spatial learning No change Morris water maze test 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Learning & memory Object recognition memory No change Novel object recognition test 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Learning & memory Spatial reference memory No change Morris water maze test 2-4 months Eadie BD , et al. (2009)
Learning & memory Spatial reference memory No change Plus-shaped water maze test 2-4 months Eadie BD , et al. (2009)
Learning & memory - Not reported - - Schtt J , et al. (2009)
Learning & memory - Not reported - - Gantois I , et al. (2005)
Maternal behavior - Not Reported - - -
Maternal behavior - Not reported - - Comery TA , et al. (1997)
Maternal behavior - Not reported - - Yau SY , et al. (2018)
Maternal behavior - Not reported - - Korb E , et al. (2017)
Maternal behavior - Not reported - - Srensen EM , et al. (2015)
Maternal behavior - Not reported - - Lim CS , et al. (2014)
Maternal behavior - Not reported - - Rogers TD , et al. (2013)
Maternal behavior - Not reported - - Michalon A , et al. (2012)
Maternal behavior - Not reported - - Rotschafer SE , et al. (2012)
Maternal behavior - Not reported - - Westmark CJ , et al. (2011)
Maternal behavior - Not reported - - Testa-Silva G , et al. (2011)
Maternal behavior - Not reported - - Mines MA , et al. (2010)
Maternal behavior - Not reported - - Dahlhaus R and El-Husseini A (2009)
Maternal behavior - Not reported - - Eadie BD , et al. (2009)
Maternal behavior - Not reported - - Schtt J , et al. (2009)
Maternal behavior - Not reported - - Gantois I , et al. (2005)
Molecular profile - Not Reported - - -
Molecular profile - Not reported - - Comery TA , et al. (1997)
Molecular profile Protein phosphorylation Increased Western blot Adult Yau SY , et al. (2018)
Molecular profile Protein localization: synapse Decreased Western blot Adult Yau SY , et al. (2018)
Molecular profile Regulation of gene expression Abnormal Rna sequencing 2.1 weeks Korb E , et al. (2017)
Molecular profile Protein-dna complex assembly Increased Quantitative pcr (qrt-pcr) 2.1 weeks Korb E , et al. (2017)
Molecular profile Protein phosphorylation Increased Western blot 2.1 weeks Korb E , et al. (2017)
Molecular profile Protein expression level evidence Increased Western blot 2.1 weeks Korb E , et al. (2017)
Molecular profile Gene expression Increased Quantitative pcr (qrt-pcr) Not specified Korb E , et al. (2017)
Molecular profile Chromatin modification: histone modification: open chromatin Increased Chromatin immunoprecipitation sequencing (chip-seq) 2.1 weeks Korb E , et al. (2017)
Molecular profile Chromatin modification No change Chromatin immunoprecipitation sequencing (chip-seq) 2.1 weeks Korb E , et al. (2017)
Molecular profile - Not reported - - Srensen EM , et al. (2015)
Molecular profile Signaling Decreased Western blot P14, P29 Lim CS , et al. (2014)
Molecular profile Protein modification process Increased Western blot P14, P29 Lim CS , et al. (2014)
Molecular profile - Not reported - - Rogers TD , et al. (2013)
Molecular profile Signaling Decreased Western blot Unreported Michalon A , et al. (2012)
Molecular profile Regulation of translation Increased Metabolic labeling of proteins Unreported Michalon A , et al. (2012)
Molecular profile - Not reported - - Rotschafer SE , et al. (2012)
Molecular profile Protein expression level evidence Increased Western blot 1 month Westmark CJ , et al. (2011)
Molecular profile - Not reported - - Testa-Silva G , et al. (2011)
Molecular profile - Not reported - - Mines MA , et al. (2010)
Molecular profile Protein expression level evidence No change Immunohistochemistry 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Molecular profile Protein expression level evidence Decreased Immunohistochemistry 2.5 - 6 months Dahlhaus R and El-Husseini A (2009)
Molecular profile - Not reported - - Eadie BD , et al. (2009)
Molecular profile - Not reported - - Schtt J , et al. (2009)
Molecular profile Transcriptome diversity No change Gene expression microarray Unreported Gantois I , et al. (2005)
Molecular profile Transcriptome diversity No change Quantitative pcr (qrt-pcr) Unreported Gantois I , et al. (2005)
Molecular profile Regulation of translation No change Quantitative pcr (qrt-pcr) Unreported Gantois I , et al. (2005)
Molecular profile Regulation of translation No change Semi-quantitative pcr (qrt-pcr) Unreported Gantois I , et al. (2005)
Molecular profile Protein expression: in situ protein expression No change Immunohistochemistry 4 months Gantois I , et al. (2005)
Molecular profile Protein expression: in situ protein expression No change Immunohistochemistry 4 months Gantois I , et al. (2005)
Molecular profile Protein expression level evidence Decreased Immunohistochemistry 10 weeks Gantois I , et al. (2005)
Molecular profile Gene expression Abnormal Semi-quantitative pcr (qrt-pcr) 10-11 weeks Gantois I , et al. (2005)
Molecular profile Gene expression Increased Quantitative pcr (qrt-pcr) 9-13 weeks Gantois I , et al. (2005)
Molecular profile Gene expression Decreased Differential display analysis of hippocampus 9-13 weeks Gantois I , et al. (2005)

M_FMR1_1_KO_HM_5HT2BAG+D1AG+PI3K INHIBITOR

Model Type: Pharmaceutical intervention
Model Genotype: Homozygous

Mutation:

Postnatal day 14 to 29 d old mice were anesthetized by an i.p. injection of 10mg/kg ketamine and 2mg/kg xylazine. A GluA1-GFP viral contruct was injected through a glass pipette, stereotaxically into the hippocampal CA1 region. For behavioral tests 10-12 injections of 6 ul high titer viral solutions were delivered along the entire hippocampus of both sides to achieve expression in one third of the CA1 pyramidal neurons. A cocktail of 5HT and Dopamine receptor agonist and antagonist was coadministered. The 5HT 2B-R agonist BW723C86 (0.05mg/kg) and 5HT 2A-R-Antagonist MDL11939 (0.01mg/kg)The D1- R agonist SKF81297 (0.05mg/kg) and D2-R antagonist Sulpiride (0.25mg/kg) were administered by four i.p. injections spaced by 4-6 h. The administration started 4-10 h before the viral injection and 16-20 h before behavioral tests. The compounds were dissolved in saline and saline was given to vehicle controls.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6

Es cell line: -
Model Source: JAX
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Lim CS , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Lim CS , et al. (2014)
Motor phenotype - Not reported - - Lim CS , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lim CS , et al. (2014)
Neurophysiology - Not reported - - Lim CS , et al. (2014)
Repetitive behavior - Not reported - - Lim CS , et al. (2014)
Seizure - Not reported - - Lim CS , et al. (2014)
Sensory - Not reported - - Lim CS , et al. (2014)
Social behavior - Not reported - - Lim CS , et al. (2014)
Communications - Not reported - - Lim CS , et al. (2014)
Developmental profile - Not reported - - Lim CS , et al. (2014)
Emotion - Not reported - - Lim CS , et al. (2014)
Immune response - Not reported - - Lim CS , et al. (2014)
Learning & memory Reward reinforced choice behavior Decreased Y-maze test P29 Lim CS , et al. (2014)
Learning & memory Cued or contextual fear conditioning Decreased Fear conditioning test P29 Lim CS , et al. (2014)
Maternal behavior - Not reported - - Lim CS , et al. (2014)
Molecular profile - Not reported - - Lim CS , et al. (2014)

M_FMR1_1_KO_HM_5HT2BAG-CA1

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Postnatal day 14 to 29 d old mice were anesthetized by an i.p. injection of 10mg/kg ketamine and 2mg/kg xylazine. A GluA1-GFP viral contruct was injected through a glass pipette, stereotaxically into the hippocampal CA1 region. For behavioral tests 10-12 injections of 6 ul high titer viral solutions were delivered along the entire hippocampus of both sides to achieve expression in one third of the CA1 pyramidal neurons. The 5HT 2B-R agonist BW723C86 (5mg/kg) and 5HT 2A-R-Antagonist MDL11939 (1mg/kg) were administered by four i.p. injections spaced by 4-6 h. The administration started 4-10 h before the viral injection and 16-20 h before behavioral tests. The compounds were dissolved in saline and saline was given to vehicle controls.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6

Es cell line: -
Model Source: JAX
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Lim CS , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Lim CS , et al. (2014)
Motor phenotype - Not reported - - Lim CS , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lim CS , et al. (2014)
Neurophysiology Rectification of ion channels Ameliorated Whole-cell patch clamp P14, P29 Lim CS , et al. (2014)
Repetitive behavior - Not reported - - Lim CS , et al. (2014)
Seizure - Not reported - - Lim CS , et al. (2014)
Sensory - Not reported - - Lim CS , et al. (2014)
Social behavior - Not reported - - Lim CS , et al. (2014)
Communications - Not reported - - Lim CS , et al. (2014)
Developmental profile - Not reported - - Lim CS , et al. (2014)
Emotion Anxiety Side effect Elevated plus maze test P29 Lim CS , et al. (2014)
Emotion Anxiety Side effect Light-dark exploration test P29 Lim CS , et al. (2014)
Immune response - Not reported - - Lim CS , et al. (2014)
Learning & memory Reward reinforced choice behavior Ameliorated Y-maze test P29 Lim CS , et al. (2014)
Learning & memory Cued or contextual fear conditioning Ameliorated Fear conditioning test P29 Lim CS , et al. (2014)
Maternal behavior - Not reported - - Lim CS , et al. (2014)
Molecular profile Protein modification process Ameliorated Western blot P14, P29 Lim CS , et al. (2014)
Molecular profile Protein modification process Ameliorated Western blot P14, P29 Lim CS , et al. (2014)

M_FMR1_1_KO_HM_5HT2BAG-L2-3

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Postnatal day 14 to 29 d old mice were anesthetized by an i.p. injection of 10mg/kg ketamine and 2mg/kg xylazine. A GluA1-GFP viral contruct was injected through a glass pipette, stereotaxically into the barrel cortex. s to achieve expression in one third of the CA1 pyramidal neurons. The 5HT 2B-R agonist BW723C86 (5mg/kg) and 5HT 2A-R-Antagonist MDL11939 (1mg/kg) were administered by four i.p. injections spaced by 4-6 h. The administration started 4-10 h before the viral injection. The compounds were dissolved in saline and saline was given to vehicle controls.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6

Es cell line: -
Model Source: JAX
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Lim CS , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Lim CS , et al. (2014)
Motor phenotype - Not reported - - Lim CS , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lim CS , et al. (2014)
Neurophysiology Rectification of ion channels Ameliorated Whole-cell patch clamp P14, P29 Lim CS , et al. (2014)
Repetitive behavior - Not reported - - Lim CS , et al. (2014)
Seizure - Not reported - - Lim CS , et al. (2014)
Sensory - Not reported - - Lim CS , et al. (2014)
Social behavior - Not reported - - Lim CS , et al. (2014)
Communications - Not reported - - Lim CS , et al. (2014)
Developmental profile - Not reported - - Lim CS , et al. (2014)
Emotion - Not reported - - Lim CS , et al. (2014)
Immune response - Not reported - - Lim CS , et al. (2014)
Learning & memory - Not reported - - Lim CS , et al. (2014)
Maternal behavior - Not reported - - Lim CS , et al. (2014)
Molecular profile - Not reported - - Lim CS , et al. (2014)

M_FMR1_1_KO_HM_5HT2BAG_D1AG

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Postnatal day 14 to 29 d old mice were anesthetized by an i.p. injection of 10mg/kg ketamine and 2mg/kg xylazine. A GluA1-GFP viral contruct was injected through a glass pipette, stereotaxically into the hippocampal CA1 region. For behavioral tests 10-12 injections of 6 ul high titer viral solutions were delivered along the entire hippocampus of both sides to achieve expression in one third of the CA1 pyramidal neurons. A cocktail of 5HT and Dopamine receptor agonist and antagonist was coadministered. The 5HT 2B-R agonist BW723C86 (0.05mg/kg) and 5HT 2A-R-Antagonist MDL11939 (0.01mg/kg)The D1- R agonist SKF81297 (0.05mg/kg) and D2-R antagonist Sulpiride (0.25mg/kg) were administered by four i.p. injections spaced by 4-6 h. The administration started 4-10 h before the viral injection and 16-20 h before behavioral tests. The compounds were dissolved in saline and saline was given to vehicle controls.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6

Es cell line: -
Model Source: JAX
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Lim CS , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Lim CS , et al. (2014)
Motor phenotype - Not reported - - Lim CS , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lim CS , et al. (2014)
Neurophysiology Rectification of ion channels Ameliorated Whole-cell patch clamp Unreported Lim CS , et al. (2014)
Repetitive behavior - Not reported - - Lim CS , et al. (2014)
Seizure - Not reported - - Lim CS , et al. (2014)
Sensory - Not reported - - Lim CS , et al. (2014)
Social behavior - Not reported - - Lim CS , et al. (2014)
Communications - Not reported - - Lim CS , et al. (2014)
Developmental profile - Not reported - - Lim CS , et al. (2014)
Emotion Anxiety No adverse effect Elevated plus maze test P29 Lim CS , et al. (2014)
Emotion Anxiety No adverse effect Light-dark exploration test P29 Lim CS , et al. (2014)
Immune response - Not reported - - Lim CS , et al. (2014)
Learning & memory Reward reinforced choice behavior Restored Y-maze test P29 Lim CS , et al. (2014)
Learning & memory Cued or contextual fear conditioning Restored Fear conditioning test P29 Lim CS , et al. (2014)
Maternal behavior - Not reported - - Lim CS , et al. (2014)
Molecular profile Signaling Ameliorated Western blot P14, P29 Lim CS , et al. (2014)
Molecular profile Protein modification process Restored Western blot P14 or 29 Lim CS , et al. (2014)

M_FMR1_1_KO_HM_APP

Model Type: RESCUE-Genetic
Model Genotype: Homozygous

Mutation:

Homologous recombination mediated deletion of exon 5 of FMR1 gene crossed with App heterozygote knockouts.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: C57BL/6

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Westmark CJ , et al. (2011)
Circadian sleep/wake cycle - Not reported - - Westmark CJ , et al. (2011)
Motor phenotype General locomotor activity Restored Open field test - Westmark CJ , et al. (2011)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Westmark CJ , et al. (2011)
Neurophysiology Synaptic plasticity Restored Field potential recordings 3 months Westmark CJ , et al. (2011)
Neurophysiology Presynaptic function: paired-pulse facilitation No adverse effect Paired-pulse ratio Unreported Westmark CJ , et al. (2011)
Repetitive behavior Repetitive digging Restored Marble-burying test 8-10 weeks Westmark CJ , et al. (2011)
Seizure Seizures Ameliorated Audiogenic seizure test P21 Westmark CJ , et al. (2011)
Sensory - Not reported - - Westmark CJ , et al. (2011)
Social behavior - Not reported - - Westmark CJ , et al. (2011)
Communications - Not reported - - Westmark CJ , et al. (2011)
Developmental profile - Not reported - - Westmark CJ , et al. (2011)
Emotion - Not reported - - Westmark CJ , et al. (2011)
Immune response - Not reported - - Westmark CJ , et al. (2011)
Learning & memory - Not reported - - Westmark CJ , et al. (2011)
Maternal behavior - Not reported - - Westmark CJ , et al. (2011)
Molecular profile Protein expression level evidence Restored Western blot 1 month Westmark CJ , et al. (2011)

M_FMR1_1_KO_HM_CTEP_A

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination mediated targeting against Exon 5 of FMR1 followed by acute treatment of CTEP of 2 mg/kg per os (s.c.) 24 hr prior.

Allele Type: Knockout
Strain of Origin: Not Specified
Genetic Background: C57BL/6

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Michalon A , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Michalon A , et al. (2012)
Motor phenotype - Not reported - - Michalon A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Michalon A , et al. (2012)
Neurophysiology Synaptic plasticity: ltd Restored Field potential recordings P25-P30 Michalon A , et al. (2012)
Repetitive behavior - Not reported - - Michalon A , et al. (2012)
Seizure Seizures Restored Acoustic startle reflex test P18-P22 Michalon A , et al. (2012)
Seizure Seizures Ameliorated Acoustic startle reflex test 1-2 months Michalon A , et al. (2012)
Sensory - Not reported - - Michalon A , et al. (2012)
Social behavior - Not reported - - Michalon A , et al. (2012)
Communications - Not reported - - Michalon A , et al. (2012)
Developmental profile - Not reported - - Michalon A , et al. (2012)
Emotion - Not reported - - Michalon A , et al. (2012)
Immune response - Not reported - - Michalon A , et al. (2012)
Learning & memory - Not reported - - Michalon A , et al. (2012)
Maternal behavior - Not reported - - Michalon A , et al. (2012)
Molecular profile - Not reported - - Michalon A , et al. (2012)

M_FMR1_1_KO_HM_CTEP_C

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination mediated targeting against Exon 5 of FMR1 followed by chronic treatment of CTEP.

Allele Type: Knockout
Strain of Origin: Not Specified
Genetic Background: C57BL/6

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Michalon A , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Michalon A , et al. (2012)
Motor phenotype Motor coordination and balance No adverse effect Accelerating rotarod test Unreported Michalon A , et al. (2012)
Motor phenotype Grip strength Refractory Grip strength test 9 weeks Michalon A , et al. (2012)
Motor phenotype General locomotor activity Restored Open field test 2-5 months Michalon A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Restored Golgi-cox staining P58-P63 Michalon A , et al. (2012)
Neurophysiology Synaptic plasticity Restored Field potential recordings P58-P65 Michalon A , et al. (2012)
Repetitive behavior - Not reported - - Michalon A , et al. (2012)
Seizure - Not reported - - Michalon A , et al. (2012)
Sensory Startle response: acoustic stimulus Restored Acoustic startle reflex test P58-P63 Michalon A , et al. (2012)
Social behavior - Not reported - - Michalon A , et al. (2012)
Communications - Not reported - - Michalon A , et al. (2012)
Developmental profile Tissue weight Ameliorated General observations 5-17 weeks Michalon A , et al. (2012)
Developmental profile Size/growth Side effect General observations Unreported Michalon A , et al. (2012)
Developmental profile Developmental trajectory Restored Irwin battery of neurological tests 9 weeks Michalon A , et al. (2012)
Emotion - Not reported - - Michalon A , et al. (2012)
Immune response - Not reported - - Michalon A , et al. (2012)
Learning & memory Cued or contextual fear conditioning: memory of cue Restored Passive avoidance test P58-P63 Michalon A , et al. (2012)
Maternal behavior - Not reported - - Michalon A , et al. (2012)
Molecular profile Signaling Restored Western blot P58-P63 Michalon A , et al. (2012)

M_FMR1_1_KO_HM_CX

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

FMR1 homozygous null mice were treated daily by intraperitoneal injections for 1 week before testing began with either DMSO alone or 5 mg/kg of CX-4945 dissolved in DMSO, and diluted 1:10 into cyclodextrin. CX-4945 is an inhibitor of the kinase that activates Brd4, casein kinase 2 (CK2).

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6J

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Korb E , et al. (2017)
Circadian sleep/wake cycle - Not reported - - Korb E , et al. (2017)
Motor phenotype General locomotor activity: ambulatory activity Ameliorated Open field test 4-8 weeks Korb E , et al. (2017)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Korb E , et al. (2017)
Neurophysiology - Not reported - - Korb E , et al. (2017)
Repetitive behavior Repetitive digging Refractory Marble-burying test 4-8 weeks Korb E , et al. (2017)
Seizure - Not reported - - Korb E , et al. (2017)
Sensory - Not reported - - Korb E , et al. (2017)
Social behavior Social approach Refractory Three-chamber social approach test 4-8 weeks Korb E , et al. (2017)
Communications - Not reported - - Korb E , et al. (2017)
Developmental profile - Not reported - - Korb E , et al. (2017)
Emotion Anxiety Refractory Open field test 5 weeks Korb E , et al. (2017)
Immune response - Not reported - - Korb E , et al. (2017)
Learning & memory - Not reported - - Korb E , et al. (2017)
Maternal behavior - Not reported - - Korb E , et al. (2017)
Molecular profile Gene expression Refractory Quantitative pcr (qrt-pcr) 4-8 weeks Korb E , et al. (2017)

M_FMR1_1_KO_HM_D1AGONIST-CA1

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Postnatal day 14 to 29 d old mice were anesthetized by an i.p. injection of 10mg/kg ketamine and 2mg/kg xylazine. A GluA1-GFP viral contruct was injected through a glass pipette, stereotaxically into the hippocampal CA1 region. For behavioral tests 10-12 injections of 6 ul high titer viral solutions were delivered along the entire hippocampus of both sides to achieve expression in one third of the CA1 pyramidal neurons. The D1- R agonist SKF81297 (5mg/kg) and D2-R antagonist Sulpiride (25mg/kg) were administered by four i.p. injections spaced by 4-6 h. The administration started 4-10 h before the viral injection and 16-20 h before behavioral tests. The compounds were dissolved in saline and saline was given to vehicle controls.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6

Es cell line: -
Model Source: JAX
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Lim CS , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Lim CS , et al. (2014)
Motor phenotype - Not reported - - Lim CS , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lim CS , et al. (2014)
Neurophysiology Rectification of ion channels Ameliorated Whole-cell patch clamp P14, P29 Lim CS , et al. (2014)
Repetitive behavior - Not reported - - Lim CS , et al. (2014)
Seizure - Not reported - - Lim CS , et al. (2014)
Sensory - Not reported - - Lim CS , et al. (2014)
Social behavior - Not reported - - Lim CS , et al. (2014)
Communications - Not reported - - Lim CS , et al. (2014)
Developmental profile - Not reported - - Lim CS , et al. (2014)
Emotion - Not reported - - Lim CS , et al. (2014)
Immune response - Not reported - - Lim CS , et al. (2014)
Learning & memory Reward reinforced choice behavior Ameliorated Y-maze test P29 Lim CS , et al. (2014)
Learning & memory Cued or contextual fear conditioning Ameliorated Fear conditioning test P29 Lim CS , et al. (2014)
Maternal behavior - Not reported - - Lim CS , et al. (2014)
Molecular profile Protein modification process Ameliorated Western blot P14, P29 Lim CS , et al. (2014)
Molecular profile Protein modification process Ameliorated Western blot P14, P29 Lim CS , et al. (2014)

M_FMR1_1_KO_HM_D1AGONIST-L2-3

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Postnatal day 14 to 29 d old mice were anesthetized by an i.p. injection of 10mg/kg ketamine and 2mg/kg xylazine. A GluA1-GFP viral contruct was injected through a glass pipette, stereotaxically into the barrel cortex. T The D1- R agonist SKF81297 (5mg/kg) and D2-R antagonist Sulpiride (25mg/kg) were administered by four i.p. injections spaced by 4-6 h. To determine the role of the PI3K/PKB signaling pathway a PI3K inhibitor LY294002 was administered at a dose of 5mg/kg. The administration started 4-10 h before the viral injection. The compounds were dissolved in saline and saline was given to vehicle controls.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Lim CS , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Lim CS , et al. (2014)
Motor phenotype - Not reported - - Lim CS , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lim CS , et al. (2014)
Neurophysiology Rectification of ion channels Ameliorated Whole-cell patch clamp P14, P29 Lim CS , et al. (2014)
Repetitive behavior - Not reported - - Lim CS , et al. (2014)
Seizure - Not reported - - Lim CS , et al. (2014)
Sensory - Not reported - - Lim CS , et al. (2014)
Social behavior - Not reported - - Lim CS , et al. (2014)
Communications - Not reported - - Lim CS , et al. (2014)
Developmental profile - Not reported - - Lim CS , et al. (2014)
Emotion - Not reported - - Lim CS , et al. (2014)
Immune response - Not reported - - Lim CS , et al. (2014)
Learning & memory - Not reported - - Lim CS , et al. (2014)
Maternal behavior - Not reported - - Lim CS , et al. (2014)
Molecular profile - Not reported - - Lim CS , et al. (2014)

M_FMR1_1_KO_HM_GLUA1CT-5HT2BAG+D1AG

Model Type: Pharmaceutical intervention
Model Genotype: Homozygous

Mutation:

Postnatal day 14 to 29 d old mice were anesthetized by an i.p. injection of 10mg/kg ketamine and 2mg/kg xylazine. A GluA1ct-GFP viral contruct was injected through a glass pipette, stereotaxically into the hippocampal CA1 region. The GluA!ct is a GFP taged cytiplasmic domain of GluA1 receptor subunit that acts as a dominant negative construct selectively blocking synaptic trafficking of endogenous or viral GluA1 contianing AMPA-rs.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Lim CS , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Lim CS , et al. (2014)
Motor phenotype - Not reported - - Lim CS , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Lim CS , et al. (2014)
Neurophysiology - Not reported - - Lim CS , et al. (2014)
Repetitive behavior - Not reported - - Lim CS , et al. (2014)
Seizure - Not reported - - Lim CS , et al. (2014)
Sensory - Not reported - - Lim CS , et al. (2014)
Social behavior - Not reported - - Lim CS , et al. (2014)
Communications - Not reported - - Lim CS , et al. (2014)
Developmental profile - Not reported - - Lim CS , et al. (2014)
Emotion - Not reported - - Lim CS , et al. (2014)
Immune response - Not reported - - Lim CS , et al. (2014)
Learning & memory Reward reinforced choice behavior Decreased Y-maze test P29 Lim CS , et al. (2014)
Learning & memory Cued or contextual fear conditioning Decreased Fear conditioning test P29 Lim CS , et al. (2014)
Maternal behavior - Not reported - - Lim CS , et al. (2014)
Molecular profile - Not reported - - Lim CS , et al. (2014)

M_FMR1_1_KO_HM_IL17a-S1DZ

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Recombinant IL17a protein (50 ng per side in 1 microliter at a rate of 180 nl/min, 7956-ML/CF, R&D) was administered directly and bilaterally into the primary somatosensory cortex dysgranular zone in adult male Fmr1 mutant four hours before testing.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6J

Es cell line: E14
Model Source: The Jackson Laboratory
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Circadian sleep/wake cycle - Not Reported - - -
Motor phenotype - Not Reported - - -
Neuroanatomy / Ultrastructure / Cytoarchitecture - Not Reported - - -
Neurophysiology - Not Reported - - -
Repetitive behavior - Not Reported - - -
Seizure - Not Reported - - -
Sensory - Not Reported - - -
Social behavior Social approach Restored Three-chamber social approach test Adult -
Communications - Not Reported - - -
Developmental profile - Not Reported - - -
Emotion - Not Reported - - -
Homeostasis - Not Reported - - -
Immune response - Not Reported - - -
Learning & memory - Not Reported - - -
Maternal behavior - Not Reported - - -
Molecular profile - Not Reported - - -

M_FMR1_1_KO_HM_JQ1-1

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

FMR1 homozygous null mice were treated daily by intraperitoneal injections for 1 week before testing began with either DMSO alone or JQ1 at 5 mg/kg, dissolved in DMSO, and diluted 1:10 into cyclodextrin.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6J

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Korb E , et al. (2017)
Circadian sleep/wake cycle - Not reported - - Korb E , et al. (2017)
Motor phenotype General locomotor activity: ambulatory activity Ameliorated Open field test 4-8 weeks Korb E , et al. (2017)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Korb E , et al. (2017)
Neurophysiology - Not reported - - Korb E , et al. (2017)
Repetitive behavior Repetitive digging Ameliorated Marble-burying test 4-8 weeks Korb E , et al. (2017)
Seizure - Not reported - - Korb E , et al. (2017)
Sensory - Not reported - - Korb E , et al. (2017)
Social behavior Social approach Refractory Three-chamber social approach test 4-8 weeks Korb E , et al. (2017)
Communications - Not reported - - Korb E , et al. (2017)
Developmental profile - Not reported - - Korb E , et al. (2017)
Emotion Anxiety Refractory Open field test 5 weeks Korb E , et al. (2017)
Immune response - Not reported - - Korb E , et al. (2017)
Learning & memory - Not reported - - Korb E , et al. (2017)
Maternal behavior - Not reported - - Korb E , et al. (2017)
Molecular profile Gene expression Ameliorated Quantitative pcr (qrt-pcr) 4-8 weeks Korb E , et al. (2017)

M_FMR1_1_KO_HM_JQ1-2

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

FMR1 homozygous null mice were treated daily by intraperitoneal injections for 1 week before testing began with either DMSO alone or JQ1 at 25 mg/kg dissolved in DMSO, and diluted 1:10 into cyclodextrin.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6J

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Korb E , et al. (2017)
Circadian sleep/wake cycle - Not reported - - Korb E , et al. (2017)
Motor phenotype General locomotor activity: ambulatory activity Restored Open field test 4-8 weeks Korb E , et al. (2017)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Korb E , et al. (2017)
Neurophysiology - Not reported - - Korb E , et al. (2017)
Repetitive behavior Repetitive digging Restored Marble-burying test 4-8 weeks Korb E , et al. (2017)
Seizure - Not reported - - Korb E , et al. (2017)
Sensory - Not reported - - Korb E , et al. (2017)
Social behavior Social approach Restored Three-chamber social approach test 4-8 weeks Korb E , et al. (2017)
Communications - Not reported - - Korb E , et al. (2017)
Developmental profile Integumentary system development No adverse effect Immunohistochemistry Not specified Korb E , et al. (2017)
Developmental profile Digestive system development No adverse effect Immunohistochemistry Not specified Korb E , et al. (2017)
Emotion Exploratory activity No adverse effect Three-chamber social approach test 4-8 weeks Korb E , et al. (2017)
Emotion Anxiety No adverse effect Open field test 4-8 weeks Korb E , et al. (2017)
Immune response Adaptive immune response: t cells No adverse effect Flow cytometric analysis Not specified Korb E , et al. (2017)
Learning & memory Object recognition memory Ameliorated Novel object recognition test 4-8 weeks Korb E , et al. (2017)
Maternal behavior - Not reported - - Korb E , et al. (2017)
Molecular profile - Not reported - - Korb E , et al. (2017)

M_FMR1_1_KO_HM_JQ1-3

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

FMR1 homozygous null mice were treated daily by intraperitoneal injections for 1 week before testing began with either DMSO alone (controls) or JQ1 at 50 mg/kg dissolved in DMSO, and diluted 1:10 into cyclodextrin.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6J

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Korb E , et al. (2017)
Circadian sleep/wake cycle - Not reported - - Korb E , et al. (2017)
Motor phenotype General locomotor activity: ambulatory activity No adverse effect Three-chamber social approach test 4.3 weeks Korb E , et al. (2017)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Korb E , et al. (2017)
Neurophysiology - Not reported - - Korb E , et al. (2017)
Repetitive behavior Repetitive digging Restored Marble-burying test 4.3 weeks Korb E , et al. (2017)
Seizure - Not reported - - Korb E , et al. (2017)
Sensory - Not reported - - Korb E , et al. (2017)
Social behavior Social approach Restored Three-chamber social approach test 4.3 weeks Korb E , et al. (2017)
Communications - Not reported - - Korb E , et al. (2017)
Developmental profile Integumentary system development Side effect Immunohistochemistry Not specified Korb E , et al. (2017)
Developmental profile Digestive system development No adverse effect Immunohistochemistry Not specified Korb E , et al. (2017)
Emotion Exploratory activity No adverse effect Three-chamber social approach test 4.3 weeks Korb E , et al. (2017)
Immune response Adaptive immune response: t cells Side effect Flow cytometric analysis Not specified Korb E , et al. (2017)
Learning & memory - Not reported - - Korb E , et al. (2017)
Maternal behavior - Not reported - - Korb E , et al. (2017)
Molecular profile Gene expression Ameliorated Quantitative pcr (qrt-pcr) Not specified Korb E , et al. (2017)

M_FMR1_1_KO_HM_JQ1_CX

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

FMR1 homozygous null mice were treated daily by intraperitoneal injections for 1 week before testing began with either DMSO alone or 5 mg/kg each of JQ1 and CX-4945 combined, dissolved in DMSO, and diluted 1:10 into cyclodextrin.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6J

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Korb E , et al. (2017)
Circadian sleep/wake cycle - Not reported - - Korb E , et al. (2017)
Motor phenotype General locomotor activity: ambulatory activity Refractory Open field test 4-8 weeks Korb E , et al. (2017)
Motor phenotype General locomotor activity: ambulatory activity Refractory Three-chamber social approach test 4-8 weeks Korb E , et al. (2017)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Restored Immunohistochemistry 4-8 weeks Korb E , et al. (2017)
Neurophysiology - Not reported - - Korb E , et al. (2017)
Repetitive behavior Repetitive digging Restored Marble-burying test 4-8 weeks Korb E , et al. (2017)
Repetitive behavior Repetitive digging Restored Marble-burying test 4-8 weeks Korb E , et al. (2017)
Seizure - Not reported - - Korb E , et al. (2017)
Sensory - Not reported - - Korb E , et al. (2017)
Social behavior Social approach Restored Three-chamber social approach test 4-8 weeks Korb E , et al. (2017)
Social behavior Social approach Restored Three-chamber social approach test 4-8 weeks Korb E , et al. (2017)
Communications - Not reported - - Korb E , et al. (2017)
Developmental profile - Not reported - - Korb E , et al. (2017)
Emotion Anxiety Restored Open field test 5 weeks Korb E , et al. (2017)
Immune response - Not reported - - Korb E , et al. (2017)
Learning & memory - Not reported - - Korb E , et al. (2017)
Maternal behavior - Not reported - - Korb E , et al. (2017)
Molecular profile Gene expression Ameliorated Quantitative pcr (qrt-pcr) 4-8 weeks Korb E , et al. (2017)
Molecular profile Gene expression Restored Quantitative pcr (qrt-pcr) 4-8 weeks Korb E , et al. (2017)

M_FMR1_1_KO_HM_LITHIUM-ACUTE

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

FMR1 KO mice were given an intraperitoneal (ip) injection of 4 mmole/kg lithium chloride (Sigma), for the acute treatment with lithium a dose that is effective in behavioral tests and increases serinephosphorylation of GSK3 in mouse brain.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Yuskaitis CJ , et al. (2009)
Circadian sleep/wake cycle - Not reported - - Yuskaitis CJ , et al. (2009)
Motor phenotype - Not reported - - Yuskaitis CJ , et al. (2009)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Yuskaitis CJ , et al. (2009)
Neurophysiology - Not reported - - Yuskaitis CJ , et al. (2009)
Repetitive behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Seizure - Not reported - - Yuskaitis CJ , et al. (2009)
Sensory - Not reported - - Yuskaitis CJ , et al. (2009)
Social behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Communications - Not reported - - Yuskaitis CJ , et al. (2009)
Developmental profile - Not reported - - Yuskaitis CJ , et al. (2009)
Emotion - Not reported - - Yuskaitis CJ , et al. (2009)
Immune response - Not reported - - Yuskaitis CJ , et al. (2009)
Learning & memory - Not reported - - Yuskaitis CJ , et al. (2009)
Maternal behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Sustained effect Western blot 3 months Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Sustained effect Western blot 3 months Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Sustained effect Western blot 3 months Yuskaitis CJ , et al. (2009)

M_FMR1_1_KO_HM_LITHIUM-CHRONIC

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

FMR1 KO mice were given water and saline ad libitum and were fed pelleted chow containing 0.2% lithium carbonate (Teklad, Madison, WI) for three weeks for the chronic treatment with lithium.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Yuskaitis CJ , et al. (2009)
Circadian sleep/wake cycle - Not reported - - Yuskaitis CJ , et al. (2009)
Motor phenotype - Not reported - - Yuskaitis CJ , et al. (2009)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Yuskaitis CJ , et al. (2009)
Neurophysiology Neurotransmitter release: neurotrophins Unexpected results Elisa 3 months Yuskaitis CJ , et al. (2009)
Repetitive behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Seizure - Not reported - - Yuskaitis CJ , et al. (2009)
Sensory - Not reported - - Yuskaitis CJ , et al. (2009)
Social behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Communications - Not reported - - Yuskaitis CJ , et al. (2009)
Developmental profile - Not reported - - Yuskaitis CJ , et al. (2009)
Emotion - Not reported - - Yuskaitis CJ , et al. (2009)
Immune response - Not reported - - Yuskaitis CJ , et al. (2009)
Learning & memory Cued or contextual fear conditioning: passive avoidance Restored Passive avoidance test 3 months Yuskaitis CJ , et al. (2009)
Maternal behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Restored Western blot 3 months Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Restored Western blot 3 months Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Restored Western blot 3 months Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Restored Western blot 3 months Yuskaitis CJ , et al. (2009)

M_FMR1_1_KO_HM_LPS

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous; hemizygous

Mutation:

FMR1 mutant mice were treated with lipopolysaccharide (50microgram/kg, i.p., acute)

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6J

Es cell line: E14
Model Source: The Jackson Laboratory
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Circadian sleep/wake cycle - Not Reported - - -
Motor phenotype - Not Reported - - -
Neuroanatomy / Ultrastructure / Cytoarchitecture - Not Reported - - -
Neurophysiology Neuronal activation Refractory Immunohistochemistry Adult -
Repetitive behavior - Not Reported - - -
Seizure - Not Reported - - -
Sensory - Not Reported - - -
Social behavior Social approach Refractory Three-chamber social approach test Adult -
Communications - Not Reported - - -
Developmental profile - Not Reported - - -
Emotion - Not Reported - - -
Homeostasis - Not Reported - - -
Immune response Cytokine levels: pro-inflammatory Side effect ELISA Adult, 2-5 months, 9-12 months -
Learning & memory - Not Reported - - -
Maternal behavior - Not Reported - - -
Molecular profile - Not Reported - - -

M_FMR1_1_KO_HM_MINOCYCLINE

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin (neo) with 30 mg/kg minocycline mother's drinking water for 28 days.

Allele Type: Knockout
Strain of Origin: Not Specified
Genetic Background: Not Specified

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Rotschafer SE , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Rotschafer SE , et al. (2012)
Motor phenotype - Not reported - - Rotschafer SE , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Rotschafer SE , et al. (2012)
Neurophysiology - Not reported - - Rotschafer SE , et al. (2012)
Repetitive behavior - Not reported - - Rotschafer SE , et al. (2012)
Seizure - Not reported - - Rotschafer SE , et al. (2012)
Sensory - Not reported - - Rotschafer SE , et al. (2012)
Social behavior - Not reported - - Rotschafer SE , et al. (2012)
Communications Ultrasonic vocalization Restored Monitoring ultrasonic vocalizations - Rotschafer SE , et al. (2012)
Communications Ultrasonic vocalization No adverse effect Monitoring ultrasonic vocalizations Unreported Rotschafer SE , et al. (2012)
Developmental profile - Not reported - - Rotschafer SE , et al. (2012)
Emotion - Not reported - - Rotschafer SE , et al. (2012)
Immune response - Not reported - - Rotschafer SE , et al. (2012)
Learning & memory - Not reported - - Rotschafer SE , et al. (2012)
Maternal behavior - Not reported - - Rotschafer SE , et al. (2012)
Molecular profile - Not reported - - Rotschafer SE , et al. (2012)

M_FMR1_1_KO_HM_MINOCYCLINE-1

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin (neo) with 30 mg/kg minocycline mother's drinking water for 28 days.

Allele Type: Knockout
Strain of Origin: Not Specified
Genetic Background: Not Specified

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Rotschafer SE , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Rotschafer SE , et al. (2012)
Motor phenotype - Not reported - - Rotschafer SE , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Rotschafer SE , et al. (2012)
Neurophysiology - Not reported - - Rotschafer SE , et al. (2012)
Repetitive behavior - Not reported - - Rotschafer SE , et al. (2012)
Seizure - Not reported - - Rotschafer SE , et al. (2012)
Sensory - Not reported - - Rotschafer SE , et al. (2012)
Social behavior - Not reported - - Rotschafer SE , et al. (2012)
Communications Ultrasonic vocalization Restored Monitoring ultrasonic vocalizations - Rotschafer SE , et al. (2012)
Communications Ultrasonic vocalization No adverse effect Monitoring ultrasonic vocalizations Unreported Rotschafer SE , et al. (2012)
Developmental profile - Not reported - - Rotschafer SE , et al. (2012)
Emotion - Not reported - - Rotschafer SE , et al. (2012)
Immune response - Not reported - - Rotschafer SE , et al. (2012)
Learning & memory - Not reported - - Rotschafer SE , et al. (2012)
Maternal behavior - Not reported - - Rotschafer SE , et al. (2012)
Molecular profile - Not reported - - Rotschafer SE , et al. (2012)

M_FMR1_1_KO_HM_MINOCYCLINE-2

Model Type: RESCUE-Pharmaceutical
Model Genotype: Hemizygous

Mutation:

Female FMR1 heterozygous mice and their pups were treated with minocycline through drinking water at 30mg/kg/day. After the offspring were weaned, minocycline administration continued in the drinking water of the offspring until they reached 2 months of age. FMR1 hemizygous knockout mice where a neomycin resistance gene is inserted into exon 5 and there is no detectable transcript or protein produced by this allele. .

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6J

Es cell line: E14
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Yau SY , et al. (2018)
Circadian sleep/wake cycle - Not reported - - Yau SY , et al. (2018)
Motor phenotype - Not reported - - Yau SY , et al. (2018)
Neuroanatomy / ultrastructure / cytoarchitecture Neuroreceptor levels: glutamate receptors: nmda receptors Restored Western blot Adult Yau SY , et al. (2018)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: dendritic tree complexity Restored Sholl analysis Adult Yau SY , et al. (2018)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: dendritic length Restored Sholl analysis Adult Yau SY , et al. (2018)
Neurophysiology Synaptic neuroreceptor ratio (nmdar/ampar) dependent transmission Restored Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology Miniature post synaptic current amplitude: excitatory Restored Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology Miniature post synaptic current amplitude: excitatory No adverse effect Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology Epsp-spike relationship Restored Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Neurophysiology Epsp-spike relationship Side effect Whole-cell voltage clamp Adult Yau SY , et al. (2018)
Repetitive behavior - Not reported - - Yau SY , et al. (2018)
Seizure - Not reported - - Yau SY , et al. (2018)
Sensory - Not reported - - Yau SY , et al. (2018)
Social behavior - Not reported - - Yau SY , et al. (2018)
Communications - Not reported - - Yau SY , et al. (2018)
Developmental profile - Not reported - - Yau SY , et al. (2018)
Emotion Exploratory activity: habituation No adverse effect Object-place recognition test Adult Yau SY , et al. (2018)
Emotion Depression No adverse effect Forced swim test Adult Yau SY , et al. (2018)
Emotion Depression No adverse effect Tail suspension test Adult Yau SY , et al. (2018)
Emotion Anxiety Side effect Open field test Adult Yau SY , et al. (2018)
Immune response - Not reported - - Yau SY , et al. (2018)
Learning & memory Spatial reference memory Restored Object-place recognition test Adult Yau SY , et al. (2018)
Learning & memory Procedural learning No adverse effect Novel object recognition test Adult Yau SY , et al. (2018)
Learning & memory Object recognition memory Restored Novel object recognition test Adult Yau SY , et al. (2018)
Maternal behavior - Not reported - - Yau SY , et al. (2018)
Molecular profile Protein phosphorylation Restored Western blot Adult Yau SY , et al. (2018)
Molecular profile Protein localization: synapse Restored Western blot Adult Yau SY , et al. (2018)

M_FMR1_1_KO_HM_MPEP

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

FMR1 KO mice were given an intraperitoneal (ip) injection of 30mg/kg MPEP (2-methyl-6-phenylethynyl-pyridine), for the acute treatment with lithium a dose that is effective in behavioral tests and increases serinephosphorylation of GSK3 in mouse brain.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Yuskaitis CJ , et al. (2009)
Circadian sleep/wake cycle - Not reported - - Yuskaitis CJ , et al. (2009)
Motor phenotype - Not reported - - Yuskaitis CJ , et al. (2009)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Yuskaitis CJ , et al. (2009)
Neurophysiology - Not reported - - Yuskaitis CJ , et al. (2009)
Repetitive behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Seizure - Not reported - - Yuskaitis CJ , et al. (2009)
Sensory - Not reported - - Yuskaitis CJ , et al. (2009)
Social behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Communications - Not reported - - Yuskaitis CJ , et al. (2009)
Developmental profile - Not reported - - Yuskaitis CJ , et al. (2009)
Emotion - Not reported - - Yuskaitis CJ , et al. (2009)
Immune response - Not reported - - Yuskaitis CJ , et al. (2009)
Learning & memory - Not reported - - Yuskaitis CJ , et al. (2009)
Maternal behavior - Not reported - - Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Restored Western blot 3 months Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Restored Western blot 3 months Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Restored Western blot 3 months Yuskaitis CJ , et al. (2009)
Molecular profile Protein phosphorylation Restored Western blot 3 months Yuskaitis CJ , et al. (2009)

M_FMR1_1_KO_HM_S1DZ-INACT

Model Type: RESCUE-Procedural
Model Genotype: Homozygous

Mutation:

Neural activity was optogenetically suppressed in the primary somatosensory cortex dysgranular zone in Fmr1 mutant mice.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: C57BL/6J

Es cell line: E14
Model Source: The Jackson Laboratory
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Circadian sleep/wake cycle - Not Reported - - -
Motor phenotype - Not Reported - - -
Neuroanatomy / Ultrastructure / Cytoarchitecture - Not Reported - - -
Neurophysiology - Not Reported - - -
Repetitive behavior - Not Reported - - -
Seizure - Not Reported - - -
Sensory - Not Reported - - -
Social behavior Social approach Restored Three-chamber social approach test Adult -
Communications - Not Reported - - -
Developmental profile - Not Reported - - -
Emotion - Not Reported - - -
Homeostasis - Not Reported - - -
Immune response - Not Reported - - -
Learning & memory - Not Reported - - -
Maternal behavior - Not Reported - - -
Molecular profile - Not Reported - - -

M_FMR1_2_KO_HM

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

FMR1 gene was generated by construction a targeting vector using homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin (neo).

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: Not Specified

Es cell line: E14
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - no authors (1994)
Physiological parameters - Not reported - - Orefice LL , et al. (2016)
Physiological parameters - Not reported - - Costa L , et al. (2012)
Physiological parameters - Not reported - - Olmos-Serrano JL , et al. (2011)
Physiological parameters - Not reported - - Shang Y , et al. (2009)
Circadian sleep/wake cycle - Not reported - - no authors (1994)
Circadian sleep/wake cycle - Not reported - - Orefice LL , et al. (2016)
Circadian sleep/wake cycle - Not reported - - Costa L , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Olmos-Serrano JL , et al. (2011)
Circadian sleep/wake cycle - Not reported - - Shang Y , et al. (2009)
Motor phenotype Spontaneous movement Increased Home cage behavior Unreported no authors (1994)
Motor phenotype - Not reported - - Orefice LL , et al. (2016)
Motor phenotype - Not reported - - Costa L , et al. (2012)
Motor phenotype General locomotor activity No change Open field test 3 months Olmos-Serrano JL , et al. (2011)
Motor phenotype General locomotor activity Increased Open field test 3 months Olmos-Serrano JL , et al. (2011)
Motor phenotype - Not reported - - Shang Y , et al. (2009)
Neuroanatomy / ultrastructure / cytoarchitecture Brain size No change Macroscopic analysis 2 months no authors (1994)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Orefice LL , et al. (2016)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Costa L , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Olmos-Serrano JL , et al. (2011)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Shang Y , et al. (2009)
Neurophysiology - Not reported - - no authors (1994)
Neurophysiology - Not reported - - Orefice LL , et al. (2016)
Neurophysiology Miniature post synaptic currents: excitatory Increased Whole-cell patch clamp P0-P1 Costa L , et al. (2012)
Neurophysiology Miniature post synaptic currents: excitatory Decreased Whole-cell patch clamp P0-P1 Costa L , et al. (2012)
Neurophysiology - Not reported - - Olmos-Serrano JL , et al. (2011)
Neurophysiology Synaptic transmission Decreased Field potential recordings Unreported Shang Y , et al. (2009)
Repetitive behavior - Not reported - - no authors (1994)
Repetitive behavior - Not reported - - Orefice LL , et al. (2016)
Repetitive behavior - Not reported - - Costa L , et al. (2012)
Repetitive behavior - Not reported - - Olmos-Serrano JL , et al. (2011)
Repetitive behavior - Not reported - - Shang Y , et al. (2009)
Seizure - Not reported - - no authors (1994)
Seizure - Not reported - - Orefice LL , et al. (2016)
Seizure - Not reported - - Costa L , et al. (2012)
Seizure - Not reported - - Olmos-Serrano JL , et al. (2011)
Seizure - Not reported - - Shang Y , et al. (2009)
Sensory - Not reported - - no authors (1994)
Sensory Touch Increased Response to air puff 6-8 weeks Orefice LL , et al. (2016)
Sensory Tactile discrimination and memory Decreased Textured novel object recognition test (t-nort) 6-8 weeks Orefice LL , et al. (2016)
Sensory Startle response: acoustic stimulus No change Acoustic startle reflex test 6-8 weeks Orefice LL , et al. (2016)
Sensory Sensorimotor gating: tactile cue Decreased Prepulse inhibition 6-8 weeks Orefice LL , et al. (2016)
Sensory Sensorimotor gating No change Prepulse inhibition 6-8 weeks Orefice LL , et al. (2016)
Sensory - Not reported - - Costa L , et al. (2012)
Sensory Startle response: acoustic stimulus Decreased Prepulse inhibition 3 months Olmos-Serrano JL , et al. (2011)
Sensory Startle response: acoustic stimulus Decreased Prepulse inhibition 3 months Olmos-Serrano JL , et al. (2011)
Sensory Sensorimotor gating No change Prepulse inhibition 3 months Olmos-Serrano JL , et al. (2011)
Sensory Sensorimotor gating Increased Prepulse inhibition 3 months Olmos-Serrano JL , et al. (2011)
Sensory - Not reported - - Shang Y , et al. (2009)
Social behavior Social interaction No change Reciprocal social interaction test Unreported no authors (1994)
Social behavior Social interaction Increased Light-dark exploration test Unreported no authors (1994)
Social behavior - Not reported - - Orefice LL , et al. (2016)
Social behavior - Not reported - - Costa L , et al. (2012)
Social behavior - Not reported - - Olmos-Serrano JL , et al. (2011)
Social behavior - Not reported - - Shang Y , et al. (2009)
Communications - Not reported - - no authors (1994)
Communications - Not reported - - Orefice LL , et al. (2016)
Communications - Not reported - - Costa L , et al. (2012)
Communications - Not reported - - Olmos-Serrano JL , et al. (2011)
Communications - Not reported - - Shang Y , et al. (2009)
Developmental profile Reproductive system development No change General observations Unreported no authors (1994)
Developmental profile Reproductive system development Decreased General observations 5-7 weeks, 2.5 months; 3.5 months; 5.5 months no authors (1994)
Developmental profile Reproductive system development Decreased Histology 5-7 weeks, 2.5 months; 3.5 months; 5.5 months no authors (1994)
Developmental profile - Not reported - - Orefice LL , et al. (2016)
Developmental profile - Not reported - - Costa L , et al. (2012)
Developmental profile - Not reported - - Olmos-Serrano JL , et al. (2011)
Developmental profile - Not reported - - Shang Y , et al. (2009)
Emotion - Not reported - - no authors (1994)
Emotion Exploratory activity No change Textured novel object recognition test (t-nort) 6-8 weeks Orefice LL , et al. (2016)
Emotion - Not reported - - Costa L , et al. (2012)
Emotion Anxiety No change Open field test 3 months Olmos-Serrano JL , et al. (2011)
Emotion - Not reported - - Shang Y , et al. (2009)
Immune response - Not reported - - no authors (1994)
Immune response - Not reported - - Orefice LL , et al. (2016)
Immune response - Not reported - - Costa L , et al. (2012)
Immune response - Not reported - - Olmos-Serrano JL , et al. (2011)
Immune response - Not reported - - Shang Y , et al. (2009)
Learning & memory Spatial learning No change Morris water maze test Unreported no authors (1994)
Learning & memory Spatial learning Decreased Morris water maze test Unreported no authors (1994)
Learning & memory Cued or contextual fear conditioning: memory of cue No change Passive avoidance test Unreported no authors (1994)
Learning & memory Object recognition memory: long-term recall Decreased Novel object recognition test 6-8 weeks Orefice LL , et al. (2016)
Learning & memory Object recognition memory No change Novel object recognition test 6-8 weeks Orefice LL , et al. (2016)
Learning & memory - Not reported - - Costa L , et al. (2012)
Learning & memory Cued or contextual fear conditioning: memory of cue Decreased Fear conditioning test 3 months Olmos-Serrano JL , et al. (2011)
Learning & memory Cued or contextual fear conditioning: memory of cue Decreased Fear conditioning test 3 months Olmos-Serrano JL , et al. (2011)
Learning & memory Cued or contextual fear conditioning: memory of context No change Fear conditioning test 3 months Olmos-Serrano JL , et al. (2011)
Learning & memory - Not reported - - Shang Y , et al. (2009)
Maternal behavior - Not reported - - no authors (1994)
Maternal behavior - Not reported - - Orefice LL , et al. (2016)
Maternal behavior - Not reported - - Costa L , et al. (2012)
Maternal behavior - Not reported - - Olmos-Serrano JL , et al. (2011)
Maternal behavior - Not reported - - Shang Y , et al. (2009)
Molecular profile - Not reported - - no authors (1994)
Molecular profile - Not reported - - Orefice LL , et al. (2016)
Molecular profile - Not reported - - Costa L , et al. (2012)
Molecular profile - Not reported - - Olmos-Serrano JL , et al. (2011)
Molecular profile - Not reported - - Shang Y , et al. (2009)

M_FMR1_2_KO_HM_CTEP-A

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination mediated targeting against Exon 5 of Fmr1 followed by acute treatment of CTEP of 2 mg/kg per os (s.c.) 24 hr prior.

Allele Type: Knockout
Strain of Origin: Not Specified
Genetic Background: C57BL/6

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Michalon A , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Michalon A , et al. (2012)
Motor phenotype - Not reported - - Michalon A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Michalon A , et al. (2012)
Neurophysiology Synaptic plasticity: ltd Restored Field potential recordings P25-P30 Michalon A , et al. (2012)
Repetitive behavior - Not reported - - Michalon A , et al. (2012)
Seizure Seizures Restored Acoustic startle reflex test P18-P22 Michalon A , et al. (2012)
Seizure Seizures Ameliorated Acoustic startle reflex test 1-2 months Michalon A , et al. (2012)
Sensory - Not reported - - Michalon A , et al. (2012)
Social behavior - Not reported - - Michalon A , et al. (2012)
Communications - Not reported - - Michalon A , et al. (2012)
Developmental profile - Not reported - - Michalon A , et al. (2012)
Emotion - Not reported - - Michalon A , et al. (2012)
Immune response - Not reported - - Michalon A , et al. (2012)
Learning & memory - Not reported - - Michalon A , et al. (2012)
Maternal behavior - Not reported - - Michalon A , et al. (2012)
Molecular profile - Not reported - - Michalon A , et al. (2012)

M_FMR1_2_KO_HM_CTEP-C

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination mediated targeting against Exon 5 of Fmr1 followed by chronic treatment of CTEP.

Allele Type: Knockout
Strain of Origin: Not Specified
Genetic Background: C57BL/6

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Michalon A , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Michalon A , et al. (2012)
Motor phenotype Motor coordination and balance No adverse effect Accelerating rotarod test Unreported Michalon A , et al. (2012)
Motor phenotype Grip strength Refractory Grip strength test 9 weeks Michalon A , et al. (2012)
Motor phenotype General locomotor activity Restored Open field test 2-5 months Michalon A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Restored Golgi-cox staining P58-P63 Michalon A , et al. (2012)
Neurophysiology Synaptic plasticity Restored Field potential recordings P58-P65 Michalon A , et al. (2012)
Repetitive behavior - Not reported - - Michalon A , et al. (2012)
Seizure - Not reported - - Michalon A , et al. (2012)
Sensory Startle response: acoustic stimulus Restored Acoustic startle reflex test P58-P63 Michalon A , et al. (2012)
Social behavior - Not reported - - Michalon A , et al. (2012)
Communications - Not reported - - Michalon A , et al. (2012)
Developmental profile Tissue weight Ameliorated General observations 5-17 weeks Michalon A , et al. (2012)
Developmental profile Size/growth Side effect General observations Unreported Michalon A , et al. (2012)
Developmental profile Developmental trajectory Restored Irwin battery of neurological tests 9 weeks Michalon A , et al. (2012)
Emotion - Not reported - - Michalon A , et al. (2012)
Immune response - Not reported - - Michalon A , et al. (2012)
Learning & memory Cued or contextual fear conditioning: memory of cue Restored Passive avoidance test P58-P63 Michalon A , et al. (2012)
Maternal behavior - Not reported - - Michalon A , et al. (2012)
Molecular profile Signaling Restored Western blot P58-P63 Michalon A , et al. (2012)

M_FMR1_3_KO_HM

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

A germline null mutation was created by crossing FMR1 mice, which has the first coding exon floxed, with mice that express Cre-recombinase in the germline (Tg(CAG-cre)13Miya). Gene inactivation was confirmed by a lack of protein expression in the brain and testes as determined by immunoblot analysis.

Allele Type: Knockout
Strain of Origin: C57BL/6J
Genetic Background: 129P2/OlaHsd * C57BL/6

Es cell line: E14
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Tyzio R , et al. (2014)
Physiological parameters - Not reported - - Mientjes EJ , et al. (2005)
Circadian sleep/wake cycle - Not reported - - Tyzio R , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Mientjes EJ , et al. (2005)
Motor phenotype - Not reported - - Tyzio R , et al. (2014)
Motor phenotype - Not reported - - Mientjes EJ , et al. (2005)
Neuroanatomy / ultrastructure / cytoarchitecture Neurotransporter expression Decreased Immunocytochemistry P15 Tyzio R , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Mientjes EJ , et al. (2005)
Neurophysiology Spontaneous post synaptic events Increased Whole-cell patch clamp P0, P15 Tyzio R , et al. (2014)
Neurophysiology Population spikes Increased Field potential recordings P15 Tyzio R , et al. (2014)
Neurophysiology Driving force of ion channel or receptor Increased Cell-attached patch clamp E20-E21, P15, P30 Tyzio R , et al. (2014)
Neurophysiology Action potential firing Increased Cell-attached patch clamp P0 Tyzio R , et al. (2014)
Neurophysiology - Not reported - - Mientjes EJ , et al. (2005)
Repetitive behavior - Not reported - - Tyzio R , et al. (2014)
Repetitive behavior - Not reported - - Mientjes EJ , et al. (2005)
Seizure - Not reported - - Tyzio R , et al. (2014)
Seizure - Not reported - - Mientjes EJ , et al. (2005)
Sensory - Not reported - - Tyzio R , et al. (2014)
Sensory - Not reported - - Mientjes EJ , et al. (2005)
Social behavior - Not reported - - Tyzio R , et al. (2014)
Social behavior - Not reported - - Mientjes EJ , et al. (2005)
Communications Ultrasonic vocalization Decreased Monitoring ultrasonic vocalizations P8 Tyzio R , et al. (2014)
Communications - Not reported - - Mientjes EJ , et al. (2005)
Developmental profile - Not reported - - Tyzio R , et al. (2014)
Developmental profile Reproductive system development Decreased Measurement of tissue weight Unreported Mientjes EJ , et al. (2005)
Developmental profile General characteristics No change General observations Unreported Mientjes EJ , et al. (2005)
Developmental profile General characteristics No change General observations Unreported Mientjes EJ , et al. (2005)
Emotion - Not reported - - Tyzio R , et al. (2014)
Emotion - Not reported - - Mientjes EJ , et al. (2005)
Immune response - Not reported - - Tyzio R , et al. (2014)
Immune response - Not reported - - Mientjes EJ , et al. (2005)
Learning & memory - Not reported - - Tyzio R , et al. (2014)
Learning & memory - Not reported - - Mientjes EJ , et al. (2005)
Maternal behavior - Not reported - - Tyzio R , et al. (2014)
Maternal behavior - Not reported - - Mientjes EJ , et al. (2005)
Molecular profile - Not reported - - Tyzio R , et al. (2014)
Molecular profile Targeted expression Decreased Immunocytochemistry Unreported Mientjes EJ , et al. (2005)
Molecular profile Targeted expression Decreased Western blot Unreported Mientjes EJ , et al. (2005)
Molecular profile Targeted expression Decreased Quantitative pcr (qrt-pcr) Unreported Mientjes EJ , et al. (2005)

M_FMR1_3_KO_HM-BUMETANIDE

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

A germline null mutation was created by crossing FMR1 mice, which has the first coding exon floxed, with mice that express Cre-recombinase in the germline (Tg(CAG-cre)13Miya). Dams were treated with Bumetanide (2-2.5 mg/kg) in drinking water, one day before delivery.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: 129P2/OlaHsd * C57BL/6

Es cell line: -
Model Source: JAX
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Tyzio R , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Tyzio R , et al. (2014)
Motor phenotype - Not reported - - Tyzio R , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Tyzio R , et al. (2014)
Neurophysiology Spontaneous post synaptic events Ameliorated Whole-cell patch clamp P15 Tyzio R , et al. (2014)
Neurophysiology Population spikes Ameliorated Field potential recordings P15 Tyzio R , et al. (2014)
Neurophysiology Driving force of ion channel or receptor Restored Cell-attached patch clamp P15 Tyzio R , et al. (2014)
Repetitive behavior - Not reported - - Tyzio R , et al. (2014)
Seizure - Not reported - - Tyzio R , et al. (2014)
Sensory - Not reported - - Tyzio R , et al. (2014)
Social behavior - Not reported - - Tyzio R , et al. (2014)
Communications Ultrasonic vocalization Restored Monitoring ultrasonic vocalizations P8 Tyzio R , et al. (2014)
Developmental profile - Not reported - - Tyzio R , et al. (2014)
Emotion - Not reported - - Tyzio R , et al. (2014)
Immune response - Not reported - - Tyzio R , et al. (2014)
Learning & memory - Not reported - - Tyzio R , et al. (2014)
Maternal behavior - Not reported - - Tyzio R , et al. (2014)
Molecular profile - Not reported - - Tyzio R , et al. (2014)

M_FMR1_3_KO_HM_BUMETANIDE

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

A germline null mutation was created by crossing Fmr1 mice, which has the first coding exon floxed, with mice that express Cre-recombinase in the germline (Tg(CAG-cre)13Miya). Dams were treated with Bumetanide (2-2.5 mg/kg) in drinking water, one day before delivery.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd
Genetic Background: 129P2/OlaHsd * C57BL/6

Es cell line: -
Model Source: JAX
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Tyzio R , et al. (2014)
Circadian sleep/wake cycle - Not reported - - Tyzio R , et al. (2014)
Motor phenotype - Not reported - - Tyzio R , et al. (2014)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Tyzio R , et al. (2014)
Neurophysiology Spontaneous post synaptic events Ameliorated Whole-cell patch clamp P15 Tyzio R , et al. (2014)
Neurophysiology Population spikes Ameliorated Field potential recordings P15 Tyzio R , et al. (2014)
Neurophysiology Driving force of ion channel or receptor Restored Cell-attached patch clamp P15 Tyzio R , et al. (2014)
Repetitive behavior - Not reported - - Tyzio R , et al. (2014)
Seizure - Not reported - - Tyzio R , et al. (2014)
Sensory - Not reported - - Tyzio R , et al. (2014)
Social behavior - Not reported - - Tyzio R , et al. (2014)
Communications Ultrasonic vocalization Restored Monitoring ultrasonic vocalizations P8 Tyzio R , et al. (2014)
Developmental profile - Not reported - - Tyzio R , et al. (2014)
Emotion - Not reported - - Tyzio R , et al. (2014)
Immune response - Not reported - - Tyzio R , et al. (2014)
Learning & memory - Not reported - - Tyzio R , et al. (2014)
Maternal behavior - Not reported - - Tyzio R , et al. (2014)
Molecular profile - Not reported - - Tyzio R , et al. (2014)

M_FMR1_4_CKO_HM_PURKINJEN

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

Conditional deletion of exon 1 of FMR1 using L7-cre, in Purkinje cells of the cerebellum

Allele Type: Conditional loss-of-function
Strain of Origin: C57BL/6J
Genetic Background: Not specified

Es cell line: E14
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Mientjes EJ , et al. (2005)
Circadian sleep/wake cycle - Not reported - - Mientjes EJ , et al. (2005)
Motor phenotype - Not reported - - Mientjes EJ , et al. (2005)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Mientjes EJ , et al. (2005)
Neurophysiology - Not reported - - Mientjes EJ , et al. (2005)
Repetitive behavior - Not reported - - Mientjes EJ , et al. (2005)
Seizure - Not reported - - Mientjes EJ , et al. (2005)
Sensory - Not reported - - Mientjes EJ , et al. (2005)
Social behavior - Not reported - - Mientjes EJ , et al. (2005)
Communications - Not reported - - Mientjes EJ , et al. (2005)
Developmental profile Reproductive system development Decreased General observations Unreported Mientjes EJ , et al. (2005)
Developmental profile General characteristics No change General observations Unreported Mientjes EJ , et al. (2005)
Emotion - Not reported - - Mientjes EJ , et al. (2005)
Immune response - Not reported - - Mientjes EJ , et al. (2005)
Learning & memory - Not reported - - Mientjes EJ , et al. (2005)
Maternal behavior - Not reported - - Mientjes EJ , et al. (2005)
Molecular profile Targeted expression Decreased Immunohistochemistry Unreported Mientjes EJ , et al. (2005)

M_FMR1_5_KI_HM

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

Knock in of transgene containing ~120 CGG.CCG repeats in the FMR1 gene with a Cre recombinase under the control of the MMTV promoter.

Allele Type: Humanized LOF mutation
Strain of Origin: Not Specified
Genetic Background: C57BL/6

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Qin M , et al. (2011)
Circadian sleep/wake cycle - Not reported - - Qin M , et al. (2011)
Motor phenotype Motor coordination and balance No change Accelerating rotarod test Unreported Qin M , et al. (2011)
Motor phenotype General locomotor activity Increased Open field test 7-9 weeks Qin M , et al. (2011)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Increased Golgi-cox staining 17 weeks Qin M , et al. (2011)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: dendritic tree complexity Decreased Golgi-cox staining 17 weeks Qin M , et al. (2011)
Neurophysiology - Not reported - - Qin M , et al. (2011)
Repetitive behavior - Not reported - - Qin M , et al. (2011)
Seizure - Not reported - - Qin M , et al. (2011)
Sensory - Not reported - - Qin M , et al. (2011)
Social behavior Social memory No change Three-chamber social approach test 8-10 weeks Qin M , et al. (2011)
Social behavior Social approach No change Three-chamber social approach test 8-10 weeks Qin M , et al. (2011)
Communications - Not reported - - Qin M , et al. (2011)
Developmental profile Size/growth No change General observations Unreported Qin M , et al. (2011)
Developmental profile Reproductive system development Decreased Measurement of tissue weight - Qin M , et al. (2011)
Developmental profile General characteristics No change General observations Unreported Qin M , et al. (2011)
Emotion Anxiety Decreased Elevated zero maze test 10-13 weeks Qin M , et al. (2011)
Emotion Anxiety Decreased Open field test 10-13 weeks Qin M , et al. (2011)
Immune response - Not reported - - Qin M , et al. (2011)
Learning & memory Cued or contextual fear conditioning: passive avoidance Decreased Passive avoidance test 13-16 weeks Qin M , et al. (2011)
Maternal behavior - Not reported - - Qin M , et al. (2011)
Molecular profile Regulation of translation Increased Autoradiographic analysis 17 weeks Qin M , et al. (2011)
Molecular profile Protein expression level evidence Decreased Western blot 3 months Qin M , et al. (2011)
Molecular profile Gene expression Increased Quantitative pcr (qrt-pcr) 3 months Qin M , et al. (2011)

M_FMR1_6_KI_HM_I304N

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

Homologous recombination mediated introduction of I304N mutation to FMR1 locus.

Allele Type: Humanized LOF mutation
Strain of Origin: Not Specified
Genetic Background: FVB x C57BL/6J

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Zang JB , et al. (2009)
Circadian sleep/wake cycle - Not reported - - Zang JB , et al. (2009)
Motor phenotype Hyperactivity No change Open field test Unreported Zang JB , et al. (2009)
Motor phenotype General locomotor activity Increased Open field test Unreported Zang JB , et al. (2009)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Zang JB , et al. (2009)
Neurophysiology - Not reported - - Zang JB , et al. (2009)
Repetitive behavior Repetitive digging Increased Marble-burying test Unreported Zang JB , et al. (2009)
Seizure - Not reported - - Zang JB , et al. (2009)
Sensory Sensorimotor gating No change Prepulse inhibition Unreported Zang JB , et al. (2009)
Sensory Pain or nociception Decreased Hot plate test Unreported Zang JB , et al. (2009)
Social behavior - Not reported - - Zang JB , et al. (2009)
Communications - Not reported - - Zang JB , et al. (2009)
Developmental profile Size/growth No change General observations Unreported Zang JB , et al. (2009)
Developmental profile Reproductive system development Increased General observations Unreported Zang JB , et al. (2009)
Developmental profile General characteristics No change General observations Unreported Zang JB , et al. (2009)
Emotion Anxiety Decreased Open field test Unreported Zang JB , et al. (2009)
Emotion Anxiety Decreased Light-dark exploration test Unreported Zang JB , et al. (2009)
Immune response - Not reported - - Zang JB , et al. (2009)
Learning & memory - Not reported - - Zang JB , et al. (2009)
Maternal behavior - Not reported - - Zang JB , et al. (2009)
Molecular profile - Not reported - - Zang JB , et al. (2009)

M_FMR1_7_KO_HM

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin, maintained in a mixed background with FVB and 129P2 corrected for eyesight by the pde6b allele.

Allele Type: Knockout
Strain of Origin: Not Specified
Genetic Background: FVB.129P2.pde6b+

Es cell line: Not Specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - -
Physiological parameters - Not reported - - Antoine MW , et al. (2019)
Physiological parameters - Not reported - - Dansie LE , et al. (2013)
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Physiological parameters - Not reported - - Rogers TD , et al. (2013)
Circadian sleep/wake cycle - Not reported - - -
Circadian sleep/wake cycle - Not reported - - Antoine MW , et al. (2019)
Circadian sleep/wake cycle - Not reported - - Dansie LE , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Rogers TD , et al. (2013)
Motor phenotype - Not reported - - -
Motor phenotype - Not reported - - Antoine MW , et al. (2019)
Motor phenotype General locomotor activity Increased Elevated plus maze test 4, 8 weeks Dansie LE , et al. (2013)
Motor phenotype General locomotor activity Increased Open field test 4, 8 weeks Dansie LE , et al. (2013)
Motor phenotype General locomotor activity Increased Open field test 10-17 weeks Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Rogers TD , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - -
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Antoine MW , et al. (2019)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dansie LE , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density No change Golgi-cox staining 11 weeks Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Increased Golgi-cox staining 11 weeks Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Rogers TD , et al. (2013)
Neurophysiology Neuronal activation No change Whole-cell voltage clamp P17-P23 -
Neurophysiology Synaptic transmission Increased Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Synaptic plasticity No change Whole-cell current clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Stimulus-spike relationship: inhibitory Decreased Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Spontaneous post synaptic event frequency: inhibitory currents No change Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Spontaneous post synaptic event frequency: excitatory currents No change Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Spontaneous post synaptic event amplitude: inhibitory currents No change Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Spontaneous post synaptic event amplitude: excitatory currents No change Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Network excitability No change Whole-cell current clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Network excitability Decreased Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Membrane potential No change Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Intrinsic membrane properties No change Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Epsp-spike relationship Decreased Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Decay kinetics of miniature post synaptic currents No change Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Decay kinetics of miniature post synaptic currents No change Whole-cell current clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Action potential properties: amplitude, rate of depolarization and repolarization No change Whole-cell current clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Action potential properties: amplitude, rate of depolarization and repolarization Increased Whole-cell voltage clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology Action potential firing Decreased Whole-cell voltage clamp P42-P92 Antoine MW , et al. (2019)
Neurophysiology Action potential firing No change Whole-cell voltage clamp 2.5 weeks - 3.14 weeks, 2 months Antoine MW , et al. (2019)
Neurophysiology Action potential firing No change Cell-attached patch clamp P17-P23 Antoine MW , et al. (2019)
Neurophysiology - Not reported - - Dansie LE , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Neurophysiology Neurotransmitter release: catecholamines Decreased In vivo amperometry Unreported Rogers TD , et al. (2013)
Repetitive behavior - Not reported - - -
Repetitive behavior - Not reported - - Antoine MW , et al. (2019)
Repetitive behavior Stereotypy Increased Open field test Unreported Dansie LE , et al. (2013)
Repetitive behavior Repetitive digging Increased Marble-burying test 4, 8 weeks, 2 months Dansie LE , et al. (2013)
Repetitive behavior Stereotypy Increased Open field test 10-17 weeks Dolan BM , et al. (2013)
Repetitive behavior Circling Increased Open field test 10-17 weeks Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Rogers TD , et al. (2013)
Seizure - Not reported - - -
Seizure - Not reported - - Antoine MW , et al. (2019)
Seizure - Not reported - - Dansie LE , et al. (2013)
Seizure Seizures Increased Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Seizure - Not reported - - Rogers TD , et al. (2013)
Sensory - Not reported - - -
Sensory Sensory-evoked response: excitation: tactile stimulus Decreased Whole-cell voltage clamp P42-P92 Antoine MW , et al. (2019)
Sensory Sensory-evoked response: excitation: tactile stimulus Decreased Whole-cell voltage clamp 2.5 weeks - 3.14 weeks, 2 months Antoine MW , et al. (2019)
Sensory - Not reported - - Antoine MW , et al. (2019)
Sensory - Not reported - - Dansie LE , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Sensory - Not reported - - Rogers TD , et al. (2013)
Social behavior - Not reported - - -
Social behavior - Not reported - - Antoine MW , et al. (2019)
Social behavior - Not reported - - Dansie LE , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Rogers TD , et al. (2013)
Communications - Not reported - - -
Communications - Not reported - - Antoine MW , et al. (2019)
Communications - Not reported - - Dansie LE , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Rogers TD , et al. (2013)
Developmental profile - Not reported - - -
Developmental profile - Not reported - - Antoine MW , et al. (2019)
Developmental profile - Not reported - - Dansie LE , et al. (2013)
Developmental profile Tissue weight Increased General observations 10-17 weeks Dolan BM , et al. (2013)
Developmental profile Size/growth No change General observations 10-17 weeks Dolan BM , et al. (2013)
Developmental profile - Not reported - - Rogers TD , et al. (2013)
Emotion - Not reported - - -
Emotion - Not reported - - Antoine MW , et al. (2019)
Emotion Anxiety Increased Elevated plus maze test 4, 8 weeks Dansie LE , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Rogers TD , et al. (2013)
Immune response - Not reported - - -
Immune response - Not reported - - Antoine MW , et al. (2019)
Immune response Adaptive immune response: b cells antibody production: igg No change Elisa Unreported Dansie LE , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Rogers TD , et al. (2013)
Learning & memory - Not reported - - -
Learning & memory - Not reported - - Antoine MW , et al. (2019)
Learning & memory - Not reported - - Dansie LE , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Rogers TD , et al. (2013)
Maternal behavior - Not reported - - -
Maternal behavior - Not reported - - Antoine MW , et al. (2019)
Maternal behavior - Not reported - - Dansie LE , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Rogers TD , et al. (2013)
Molecular profile - Not reported - - -
Molecular profile - Not reported - - Antoine MW , et al. (2019)
Molecular profile Protein phosphorylation Increased Elisa Unreported Dansie LE , et al. (2013)
Molecular profile Protein phosphorylation Decreased Elisa Unreported Dansie LE , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Rogers TD , et al. (2013)

M_FMR1_7_KO_HM_FRAX486-H

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 30 mg/kg for 8 hours.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Restored Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486-L-1

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 10 mg/kg for 8 hours.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Ameliorated Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486-L-2

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 10 mg/kg for 5 days and tested 8 hrs after final dose.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Ameliorated Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486-M-1

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 20 mg/kg for 8 hours.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density No adverse effect Golgi-cox staining 11 weeks Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Restored Golgi-cox staining 11 weeks Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Ameliorated Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486-M-3

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 20 mg/kg for 5 days and tested 8 hours after final dose.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype Motor coordination and balance No adverse effect Accelerating rotarod test 10-17 weeks Dolan BM , et al. (2013)
Motor phenotype General locomotor activity Restored Open field test 10-17 weeks Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior Stereotypy Restored Open field test 10-17 weeks Dolan BM , et al. (2013)
Repetitive behavior Circling Restored Open field test 10-17 weeks Dolan BM , et al. (2013)
Seizure Seizures Restored Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile Tissue weight Refractory General observations 10-17 weeks Dolan BM , et al. (2013)
Developmental profile Size/growth No adverse effect General observations 10-17 weeks Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486_H

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 30 mg/kg for 8 hours.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Restored Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486_L

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 10 mg/kg for 8 hours.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Ameliorated Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486_L_D

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 10 mg/kg for 5 days and tested 8 hrs after final dose.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Ameliorated Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486_M

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 20 mg/kg for 8 hours.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density No adverse effect Golgi-cox staining 11 weeks Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Restored Golgi-cox staining 11 weeks Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Ameliorated Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486_M-2

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 20 mg/kg for one dose and tested 5 days after.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Refractory Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486_M_D

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 20 mg/kg for 5 days and tested 8 hours after final dose.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype Motor coordination and balance No adverse effect Accelerating rotarod test 10-17 weeks Dolan BM , et al. (2013)
Motor phenotype General locomotor activity Restored Open field test 10-17 weeks Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior Stereotypy Restored Open field test 10-17 weeks Dolan BM , et al. (2013)
Repetitive behavior Circling Restored Open field test 10-17 weeks Dolan BM , et al. (2013)
Seizure Seizures Restored Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile Tissue weight Refractory General observations 10-17 weeks Dolan BM , et al. (2013)
Developmental profile Size/growth No adverse effect General observations 10-17 weeks Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_FRAX486_M_S

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of FRAX486 at 20 mg/kg for one dose and tested 5 days after.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dolan BM , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dolan BM , et al. (2013)
Motor phenotype - Not reported - - Dolan BM , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dolan BM , et al. (2013)
Neurophysiology - Not reported - - Dolan BM , et al. (2013)
Repetitive behavior - Not reported - - Dolan BM , et al. (2013)
Seizure Seizures Refractory Audiogenic seizure test 10-17 weeks Dolan BM , et al. (2013)
Sensory - Not reported - - Dolan BM , et al. (2013)
Social behavior - Not reported - - Dolan BM , et al. (2013)
Communications - Not reported - - Dolan BM , et al. (2013)
Developmental profile - Not reported - - Dolan BM , et al. (2013)
Emotion - Not reported - - Dolan BM , et al. (2013)
Immune response - Not reported - - Dolan BM , et al. (2013)
Learning & memory - Not reported - - Dolan BM , et al. (2013)
Maternal behavior - Not reported - - Dolan BM , et al. (2013)
Molecular profile - Not reported - - Dolan BM , et al. (2013)

M_FMR1_7_KO_HM_MINOCYCLINE

Model Type: RESCUE-Pharmaceutical
Model Genotype: Homozygous

Mutation:

Homologous recombination against Exon 5 which was interrupted by the positive selection marker gene neomycin followed by administration of minocycline at 30 mg/kg/day in drinking water for 4 or 8 weeks.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: FVB

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Dansie LE , et al. (2013)
Circadian sleep/wake cycle - Not reported - - Dansie LE , et al. (2013)
Motor phenotype General locomotor activity Restored Elevated plus maze test 4, 8 weeks Dansie LE , et al. (2013)
Motor phenotype General locomotor activity Restored Elevated plus maze test 4, 8 weeks Dansie LE , et al. (2013)
Motor phenotype General locomotor activity Restored Open field test 4, 8 weeks Dansie LE , et al. (2013)
Motor phenotype General locomotor activity Restored Open field test 4, 8 weeks Dansie LE , et al. (2013)
Motor phenotype General locomotor activity Refractory Open field test >2 months Dansie LE , et al. (2013)
Motor phenotype General locomotor activity Restored Open field test >2 months Dansie LE , et al. (2013)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Dansie LE , et al. (2013)
Neurophysiology - Not reported - - Dansie LE , et al. (2013)
Repetitive behavior Repetitive digging Refractory Marble-burying test >2 months Dansie LE , et al. (2013)
Repetitive behavior Repetitive digging Restored Marble-burying test >2 months Dansie LE , et al. (2013)
Repetitive behavior Repetitive digging Restored Marble-burying test 4, 8 weeks Dansie LE , et al. (2013)
Repetitive behavior Repetitive digging Restored Marble-burying test 4, 8 weeks; 2 months Dansie LE , et al. (2013)
Seizure Seizures Ameliorated Audiogenic seizure test 4 weeks Dansie LE , et al. (2013)
Sensory - Not reported - - Dansie LE , et al. (2013)
Social behavior - Not reported - - Dansie LE , et al. (2013)
Communications - Not reported - - Dansie LE , et al. (2013)
Developmental profile - Not reported - - Dansie LE , et al. (2013)
Emotion Anxiety Restored Elevated plus maze test 4, 8 weeks Dansie LE , et al. (2013)
Emotion Anxiety Restored Elevated plus maze test 4, 8 weeks Dansie LE , et al. (2013)
Immune response Adaptive immune response: b cells No adverse effect Elisa 10 weeks Dansie LE , et al. (2013)
Learning & memory - Not reported - - Dansie LE , et al. (2013)
Maternal behavior - Not reported - - Dansie LE , et al. (2013)
Molecular profile Protein phosphorylation Restored Elisa >4 weeks Dansie LE , et al. (2013)
Molecular profile Protein phosphorylation Refractory Elisa >4 weeks Dansie LE , et al. (2013)

M_FMR1_8_KO_HM

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

FMR1 KO mice were originally on the C57Bl/6 background but to generate phenotyped in a mixed C57BL/6 129SveJ background form litters.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6.129SVeJ

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Bhattacharya A , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Bhattacharya A , et al. (2012)
Motor phenotype Motor coordination and balance Decreased Accelerating rotarod test Unreported Bhattacharya A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Increased Golgi-cox staining 3-4 months Bhattacharya A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Abnormal Golgi-cox staining 3-4 months Bhattacharya A , et al. (2012)
Neurophysiology Synaptic plasticity: ltd Increased Whole-cell patch clamp 3-5 weeks Bhattacharya A , et al. (2012)
Repetitive behavior Stereotypy Increased Marble-burying test Unreported Bhattacharya A , et al. (2012)
Seizure - Not reported - - Bhattacharya A , et al. (2012)
Sensory - Not reported - - Bhattacharya A , et al. (2012)
Social behavior Social memory Decreased Three-chamber social approach test Unreported Bhattacharya A , et al. (2012)
Social behavior Social interaction Increased Open field test Unreported Bhattacharya A , et al. (2012)
Communications - Not reported - - Bhattacharya A , et al. (2012)
Developmental profile Size/growth Increased General observations 6 weeks Bhattacharya A , et al. (2012)
Developmental profile Reproductive system development Decreased General observations 3-4 months Bhattacharya A , et al. (2012)
Emotion - Not reported - - Bhattacharya A , et al. (2012)
Immune response - Not reported - - Bhattacharya A , et al. (2012)
Learning & memory Spatial working memory No change Water y-maze test Unreported Bhattacharya A , et al. (2012)
Learning & memory Spatial working memory Decreased Water y-maze test Unreported Bhattacharya A , et al. (2012)
Learning & memory Object recognition memory Decreased Novel object recognition test Unreported Bhattacharya A , et al. (2012)
Maternal behavior - Not reported - - Bhattacharya A , et al. (2012)
Molecular profile Regulation of translation Increased Surface sensing of translation (sunset) Unreported Bhattacharya A , et al. (2012)
Molecular profile Protein phosphorylation Increased Western blot Unreported Bhattacharya A , et al. (2012)
Molecular profile Protein modification process Increased Immunohistochemistry 8-10 weeks Bhattacharya A , et al. (2012)
Molecular profile Protein expression level evidence No change Western blot Unreported Bhattacharya A , et al. (2012)
Molecular profile Protein expression level evidence Increased Western blot Unreported Bhattacharya A , et al. (2012)

M_FMR1_8_KO_HM_S6K1_DM-HM

Model Type: RESCUE-Genetic
Model Genotype: Homozygous

Mutation:

FMR1 KO were crossed with homozygous S6K1 KO mice to produce double knockout, maintained in the mixed background. As they used males, FMR1 was hemizygous.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: C57BL/6.129SVeJ

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Bhattacharya A , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Bhattacharya A , et al. (2012)
Motor phenotype Motor coordination and balance Restored Accelerating rotarod test 4-6 months Bhattacharya A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Restored Golgi-cox staining P90-P100 Bhattacharya A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture Dendritic architecture: spine density Restored Golgi-cox staining P90-P100 Bhattacharya A , et al. (2012)
Neurophysiology Synaptic plasticity: ltd Restored Whole-cell patch clamp P23-P35 Bhattacharya A , et al. (2012)
Neurophysiology Presynaptic function: paired-pulse facilitation No adverse effect Whole-cell patch clamp 3-5 weeks Bhattacharya A , et al. (2012)
Repetitive behavior Stereotypy Refractory Marble-burying test 4-6 months Bhattacharya A , et al. (2012)
Seizure - Not reported - - Bhattacharya A , et al. (2012)
Sensory - Not reported - - Bhattacharya A , et al. (2012)
Social behavior Social interaction Refractory Open field test 4-6 months Bhattacharya A , et al. (2012)
Social behavior Social approach Restored Three-chamber social approach test 4-6 months Bhattacharya A , et al. (2012)
Communications - Not reported - - Bhattacharya A , et al. (2012)
Developmental profile Size/growth Restored General observations P28-P84 Bhattacharya A , et al. (2012)
Developmental profile General characteristics No adverse effect General observations Unreported Bhattacharya A , et al. (2012)
Developmental profile Developmental trajectory Restored General observations P90-P100 Bhattacharya A , et al. (2012)
Emotion - Not reported - - Bhattacharya A , et al. (2012)
Immune response - Not reported - - Bhattacharya A , et al. (2012)
Learning & memory Spatial working memory Restored Water y-maze test 4-6 months Bhattacharya A , et al. (2012)
Learning & memory Spatial working memory No adverse effect Water y-maze test 4-6 months Bhattacharya A , et al. (2012)
Learning & memory Object recognition memory Restored Novel object recognition test 4-6 months Bhattacharya A , et al. (2012)
Maternal behavior - Not reported - - Bhattacharya A , et al. (2012)
Molecular profile Regulation of translation Restored Surface sensing of translation (sunset) 4-6 weeks Bhattacharya A , et al. (2012)
Molecular profile Protein phosphorylation Restored Western blot 2 months Bhattacharya A , et al. (2012)
Molecular profile Protein phosphorylation Restored Immunohistochemistry 8-10 weeks Bhattacharya A , et al. (2012)
Molecular profile Protein expression level evidence Refractory Western blot 2 months Bhattacharya A , et al. (2012)
Molecular profile Protein expression level evidence Restored Western blot 2 months Bhattacharya A , et al. (2012)

M_FMR1_8_KO_HM_S6K1_DM-HT

Model Type: RESCUE-Genetic
Model Genotype: Homozygous

Mutation:

FMR1 KO were crossed with homozygous S6K1 KO mice to produce double knockout, maintained in the mixed background. As they used males, FMR1 was hemizygous.

Allele Type: Knockout
Strain of Origin: Not specified
Genetic Background: C57BL/6.129SVeJ

Es cell line: Not specified
Model Source: Not Specified
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Bhattacharya A , et al. (2012)
Circadian sleep/wake cycle - Not reported - - Bhattacharya A , et al. (2012)
Motor phenotype - Not reported - - Bhattacharya A , et al. (2012)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Bhattacharya A , et al. (2012)
Neurophysiology Synaptic plasticity: ltd Refractory Whole-cell patch clamp P23-P35 Bhattacharya A , et al. (2012)
Repetitive behavior - Not reported - - Bhattacharya A , et al. (2012)
Seizure - Not reported - - Bhattacharya A , et al. (2012)
Sensory - Not reported - - Bhattacharya A , et al. (2012)
Social behavior - Not reported - - Bhattacharya A , et al. (2012)
Communications - Not reported - - Bhattacharya A , et al. (2012)
Developmental profile - Not reported - - Bhattacharya A , et al. (2012)
Emotion - Not reported - - Bhattacharya A , et al. (2012)
Immune response - Not reported - - Bhattacharya A , et al. (2012)
Learning & memory - Not reported - - Bhattacharya A , et al. (2012)
Maternal behavior - Not reported - - Bhattacharya A , et al. (2012)
Molecular profile Protein phosphorylation Refractory Western blot 3-4 months Bhattacharya A , et al. (2012)

M_FMR1_9_KO_HM

Model Type: Genetic
Model Genotype: Homozygous

Mutation:

FMR1 KO mice, floxed at exon 5, maintained in the mixed background of C57Bl/6 and 129P2.

Allele Type: Knockout
Strain of Origin: -
Genetic Background: C57BL/6.129P2

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Selby L , et al. (2007)
Circadian sleep/wake cycle - Not reported - - Selby L , et al. (2007)
Motor phenotype - Not reported - - Selby L , et al. (2007)
Neuroanatomy / ultrastructure / cytoarchitecture Neuronal number: interneurons Decreased Immunohistochemistry 12 months Selby L , et al. (2007)
Neuroanatomy / ultrastructure / cytoarchitecture Brain morphology No change Immunohistochemistry 12 months Selby L , et al. (2007)
Neuroanatomy / ultrastructure / cytoarchitecture Brain morphology No change Immunohistochemistry 12 months Selby L , et al. (2007)
Neuroanatomy / ultrastructure / cytoarchitecture Brain morphology Increased Immunohistochemistry 12 months Selby L , et al. (2007)
Neuroanatomy / ultrastructure / cytoarchitecture Brain morphology Decreased Immunohistochemistry 12 months Selby L , et al. (2007)
Neuroanatomy / ultrastructure / cytoarchitecture Brain cytoarchitecture Increased Cell size measurement 12 months Selby L , et al. (2007)
Neurophysiology - Not reported - - Selby L , et al. (2007)
Repetitive behavior - Not reported - - Selby L , et al. (2007)
Seizure - Not reported - - Selby L , et al. (2007)
Sensory - Not reported - - Selby L , et al. (2007)
Social behavior - Not reported - - Selby L , et al. (2007)
Communications - Not reported - - Selby L , et al. (2007)
Developmental profile - Not reported - - Selby L , et al. (2007)
Emotion - Not reported - - Selby L , et al. (2007)
Immune response - Not reported - - Selby L , et al. (2007)
Learning & memory - Not reported - - Selby L , et al. (2007)
Maternal behavior - Not reported - - Selby L , et al. (2007)
Molecular profile Protein expression level evidence Increased Immunohistochemistry 12 months Selby L , et al. (2007)
Molecular profile Protein expression level evidence Decreased Immunohistochemistry 12 months Selby L , et al. (2007)

M_SYNGAP1_9_KO_FMR1_DM

Model Type: Genetic
Model Genotype: Hemizygous (Fmr1), heterozygous (Syngap1)

Mutation:

Syngap1 het mice were mated with FMR1-/+ females to get FMR1-/y:Syngap1+/- mice.

Allele Type: Knockout
Strain of Origin: 129P2/OlaHsd*C57BL/6JOla
Genetic Background: -

Es cell line: -
Model Source: -
Phenotypic Profile
Category Entity Value Change Experimental Paradigm Age at Testing Author, Year
Physiological parameters - Not reported - - Barnes SA , et al. (2015)
Circadian sleep/wake cycle - Not reported - - Barnes SA , et al. (2015)
Motor phenotype - Not reported - - Barnes SA , et al. (2015)
Neuroanatomy / ultrastructure / cytoarchitecture - Not reported - - Barnes SA , et al. (2015)
Neurophysiology Synaptic plasticity: hippocampal ltd Increased Field potential recordings 3-4.5 weeks Barnes SA , et al. (2015)
Repetitive behavior - Not reported - - Barnes SA , et al. (2015)
Seizure - Not reported - - Barnes SA , et al. (2015)
Sensory - Not reported - - Barnes SA , et al. (2015)
Social behavior - Not reported - - Barnes SA , et al. (2015)
Communications - Not reported - - Barnes SA , et al. (2015)
Developmental profile - Not reported - -