Target intelligence / Profile preview

Atonal bHLH transcription factor 7 (ATOH7)

Target
ATOH7
Molecular classification
Transcription factor, Basic helix-loop-helix (bHLH) protein, Proneural transcription factor
01

Overview

Atonal bHLH transcription factor 7 (ATOH7) is a proneural basic helix-loop-helix transcription factor essential for the genesis of retinal ganglion cells (RGCs), which are the primary projection neurons of the retina whose axons form the optic nerve[1][2][3]. ATOH7 is dynamically and transiently expressed in multipotent retinal progenitor cells during a critical window in eye development preceding RGC differentiation[2][3]. It binds enhancer and promoter DNA sites (notably the sequence 5'-CAG[GC]TG-3'), sometimes as a dimer, to initiate a transcriptional program determining RGC fate and regulating axon targeting, optic nerve formation, and circadian photoentrainment in the retina[3]. Mutations or regulatory deletions affecting ATOH7 can cause congenital diseases such as nonsyndromic congenital retinal nonattachment, optic nerve aplasia, and persistent hyperplastic primary vitreous[1][3]. While critical for normal development, there is currently no evidence that ATOH7 is a direct therapeutic target for drugs or other therapies, and its disruption leads mainly to developmental anomalies rather than issues with postnatal pharmacological manipulation[1][3].

Other names
ATOH7ATH5BHLHA13bHLHa13Math5Atonal bHLH transcription factor 7Class A basic helix-loop-helix protein 13Protein atonal homolog 7NCRNAPHPVARRNANChelix-loop-helix protein hATH-5transcription factor ATOH7atonal homolog 7atonal homolog bHLH transcription factor 7
02

Biological functions

Retinal ganglion cell (RGC) genesis and specificationRegulation of neurogenesis in the retinaDNA binding and transcription regulationRetinal circadian rhythm photoentrainmentBrainstem auditory signaling and binaural processing
03

Disease associations

Congenital retinal nonattachment (NCRNA)Persistent hyperplastic primary vitreous (PHPV)Optic nerve hypoplasiaVitreoretinal dystrophyOther congenital diseases of optic nerve/retina
04

Safety considerations

Loss of ATOH7 function during development leads to optic nerve aplasia and blindness, but there are no reported safety concerns for therapeutics as this is not a current drug target[1][3].Disruption can result in severe, non-compensable congenital retinal anomalies
05

Biomarkers

Potential biomarker for congenital retinal nonattachmentRetina development competence states in research

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