ATOH1

Chr 4AD

atonal bHLH transcription factor 1

Also known as: ATH1, DFNA89, HATH1, MATH-1, bHLHa14

Enables sequence-specific double-stranded DNA binding activity. Predicted to be involved in several processes, including neuron differentiation; positive regulation of neuron differentiation; and positive regulation of transcription by RNA polymerase II. Predicted to act upstream of or within several processes, including generation of neurons; neuroblast migration; and positive regulation of inner ear auditory receptor cell differentiation. Predicted to be located in chromatin. Predicted to be active in nucleus. Implicated in autosomal dominant nonsyndromic deafness 89. [provided by Alliance of Genome Resources, Apr 2025]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

?Deafness, autosomal dominant 89MIM #620284
AD
0
Active trials
70
Pubs (1 yr)
P/LP submissions
P/LP missense
1.05
LOEUF
LOF
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.05LOEUF
pLI 0.018
Z-score 1.48
OE 0.46 (0.231.05)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.13Z-score
OE missense 1.03 (0.921.15)
215 obs / 209.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.46 (0.231.05)
00.351.4
Missense OE?1.03 (0.921.15)
00.61.4
Synonymous OE?1.19
01.21.6
LoF obs/exp: 4 / 8.7Missense obs/exp: 215 / 209.5Syn Z: -1.46

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ATOH1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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