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CHD4

Domain

The KIKL motif recognizes and binds the NET domain of BRD3.

Function

ATP-dependent helicase that binds and distorts nucleosomal DNA (PubMed:28977666, PubMed:32543371). Acts as a component of the histone deacetylase NuRD complex which participates in the remodeling of chromatin (PubMed:16428440, PubMed:17626165, PubMed:28977666, PubMed:9804427). Localizes to acetylated damaged chromatin in a ZMYND8-dependent manner, to promote transcriptional repression and double-strand break repair by homologous recombination (PubMed:25593309). Involved in neurogenesis (By similarity).

Involvement in disease

Sifrim-Hitz-Weiss syndrome

SIHIWES

An autosomal dominant syndrome characterized by intellectual disability, variable congenital defects affecting cardiac, skeletal, and urogenital systems. Short stature, macrocephaly, hearing impairment, and facial dysmorphism are present in some patients.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the SNF2/RAD54 helicase family.

Tissue Specificity

Widely expressed.

Cellular localization

Alternative names

Chromodomain-helicase-DNA-binding protein 4, CHD-4, ATP-dependent helicase CHD4, Mi-2 autoantigen 218 kDa protein, Mi2-beta, CHD4

swissprot:Q14839 entrezGene:1108 omim:603277