Human Gene Module / Chromosome 19 / ZNF865

ZNF865zinc finger protein 865

SFARI Gene Score
1
High Confidence Criteria 1.1
Autism Reports / Total Reports
4 / 5
Rare Variants / Common Variants
22 / 0
Aliases
-
Associated Syndromes
-
Chromosome Band
19q13.42
Associated Disorders
-
Relevance to Autism

Bradbrook et al., 2025 described a cohort of 18 patients with protein-truncating variants in the ZNF865 gene (the majority of which were de novo in origin and clustered toward the C-terminus) presenting with a neurodevelopmental disorder characterized by speech delay, cognitive delay or intellectual disability, hypotonia, brain MRI abnormalities, and dysmorphic features; seven patients were reported to have been diagnosed with autism spectrum disorder, with two additional patients having autistic features without a confirmed diagnosis. Additional de novo variants in ZNF865, including two protein-truncating variants and four missense variants, have been reported in ASD probands from the Simons Simplex Collection, SPARK, the Autism Sequencing Consortium, MSSNG, and a Chinese ASD cohort (Satterstrom et al., 2020; Zhou et al., 2022; Wang et al., 2023).

Molecular Function

Predicted to enable DNA-binding transcription factor activity and RNA polymerase II cis-regulatory region sequence-specific DNA binding activity. Predicted to be involved in regulation of transcription by RNA polymerase II. Predicted to be located in nucleus.

SFARI Genomic Platforms
Reports related to ZNF865 (5 Reports)
# Type Title Author, Year Autism Report Associated Disorders
1 Support Large-Scale Exome Sequencing Study Implicates Both Developmental and Functional Changes in the Neurobiology of Autism Satterstrom FK et al. (2020) Yes -
2 Support - Zhou X et al. (2022) Yes -
3 Support - Wang J et al. (2023) Yes -
4 Primary - Samuel M Bradbrook et al. (2026) No ASD, epilepsy/seizures
5 Support - Richard G Boles et al. (2025) Yes ID
Rare Variants   (22)
Status Allele Change Residue Change Variant Type Inheritance Pattern Parental Transmission Family Type PubMed ID Author, Year
c.2015C>A p.Ser672Ter stop_gained De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.2091C>G p.Tyr697Ter stop_gained De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.2141G>A p.Trp714Ter stop_gained De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.2142G>A p.Trp714Ter stop_gained De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.1381G>T p.Ala461Ser missense_variant De novo - Simplex 35982159 Zhou X et al. (2022)
c.2629C>G p.Arg877Gly missense_variant De novo - Simplex 37393044 Wang J et al. (2023)
c.1999C>T p.Leu667= synonymous_variant De novo - Simplex 35982159 Zhou X et al. (2022)
c.539C>T p.Pro180Leu missense_variant De novo - Multiplex 35982159 Zhou X et al. (2022)
c.1256C>T p.Ala419Val missense_variant De novo - - 31981491 Satterstrom FK et al. (2020)
c.1718C>T p.Thr573Met missense_variant De novo - - 41010044 Richard G Boles et al. (2025)
c.2304_2305delTG p.Gly770Ter stop_gained De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.2038del p.Asp680IlefsTer6 frameshift_variant De novo - Simplex 35982159 Zhou X et al. (2022)
c.2952del p.Pro985ArgfsTer97 frameshift_variant De novo - Simplex 35982159 Zhou X et al. (2022)
c.2395C>T p.Gln799Ter stop_gained Unknown Not maternal - 40936200 Samuel M Bradbrook et al. (2026)
c.194del p.Pro65ArgfsTer98 frameshift_variant De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.1616del p.Gly539GlufsTer27 frameshift_variant De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.2339del p.Gly780ValfsTer29 frameshift_variant De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.2351del p.Gly784ValfsTer25 frameshift_variant De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.2363del p.Gly788AlafsTer21 frameshift_variant De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.1951del p.Ala651ProfsTer17 frameshift_variant Familial - - 40936200 Samuel M Bradbrook et al. (2026)
c.2398_2399del p.Ser800PhefsTer163 frameshift_variant De novo - - 40936200 Samuel M Bradbrook et al. (2026)
c.1573_1574dup p.His526ProfsTer41 frameshift_variant Unknown Not maternal - 40936200 Samuel M Bradbrook et al. (2026)
Common Variants  

No common variants reported.

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