Human Gene Module / Chromosome 19 / TBCB

TBCBtubulin folding cofactor B

SFARI Gene Score
3
Suggestive Evidence Criteria 3.1
Autism Reports / Total Reports
3 / 3
Rare Variants / Common Variants
4 / 0
Aliases
-
Associated Syndromes
-
Chromosome Band
19q13.12
Associated Disorders
-
Relevance to Autism

Morag et al., 2025 described a cohort of ten individuals from eight families of Ashkenazi descent, all with the same homozygous missense variant in the TBCB gene (p.Tyr197Asn) and presenting with a neurodevelopmental disorder characterized by global developmental delay, autism spectrum disorder, and late childhood-onset spastic paraparesis; patient-derived fibroblasts displayed reduced TBCB expression. Additional functional assessment of the p.Tyr197Asn variant using the S. cerevisiae orthologue ALF1 found that mutant ALF1 resulted in increased benomyl sensitivity in yeast, resembling a loss-of-function phenotype, while the homologous mutant in Drosophila led to reduced survival and impaired climbing ability. De novo missense variants in TBCB have also been identified in ASD probands from the Simons Simplex Collection and the SPARK cohort (Iossifov et al., 2014; Zhou et al., 2022).

Molecular Function

Predicted to enable microtubule plus-end binding activity. Predicted to be involved in cytoplasmic microtubule organization. Located in cytosol.

SFARI Genomic Platforms
Reports related to TBCB (3 Reports)
# Type Title Author, Year Autism Report Associated Disorders
1 Support The contribution of de novo coding mutations to autism spectrum disorder Iossifov I et al. (2014) Yes -
2 Support - Zhou X et al. (2022) Yes -
3 Primary - Sharon Bratman Morag et al. () Yes ADHD
Rare Variants   (4)
Status Allele Change Residue Change Variant Type Inheritance Pattern Parental Transmission Family Type PubMed ID Author, Year
c.23C>A p.Ala8Glu missense_variant De novo - Simplex 35982159 Zhou X et al. (2022)
c.587G>A p.Arg196His missense_variant De novo - Simplex 25363768 Iossifov I et al. (2014)
c.589T>A p.Tyr197Asn missense_variant Familial Both parents Simplex 40856104 Sharon Bratman Morag et al. ()
c.589T>A p.Tyr197Asn missense_variant Familial Both parents Multiplex 40856104 Sharon Bratman Morag et al. ()
Common Variants  

No common variants reported.

Submit New Gene

Report an Error