ZMYND11

Professionals

ZMYND11 (encoding zinc-finger MYND domain 11) is a tumor suppressor gene whose corresponding protein recognizes chromatin trimethylated at lysine 36 of histone H3.3 (H3.3K36me3) and regulates elongation by RNA polymerase II. It is associated with highly expressed genes and might be an important transcriptional corepressor early in development. Additionally, ZMYND11 has been demonstrated to have an inhibitory role in neuronal differentiation. This autosomal dominant syndrome has a phenotype which includes dysmorphic facial features including hypertelorism and wide mouth, the neurodevelopmental phenotype includes epilepsy, autism spectrum disorders, intellectual disability, aggression, and complex neuropsychiatric features.