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ATOH1

Function

Transcriptional regulator. Activates E box-dependent transcription in collaboration with TCF3/E47, but the activity is completely antagonized by the negative regulator of neurogenesis HES1. Plays a role in the differentiation of subsets of neural cells by activating E box-dependent transcription (By similarity).

Involvement in disease

Deafness, autosomal dominant, 89

DFNA89

An autosomal dominant form of non-syndromic deafness characterized by progressive hearing loss, with onset at birth or early childhood.

None

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

Cellular localization

Alternative names

ATH1, BHLHA14, ATOH1, Transcription factor ATOH1, Atonal bHLH transcription factor 1, Class A basic helix-loop-helix protein 14, Helix-loop-helix protein hATH-1, Protein atonal homolog 1, bHLHa14, hATH1

swissprot:Q92858 entrezGene:474 omim:601461