HES4

Chr 1

hes family bHLH transcription factor 4

Also known as: bHLHb42

HES4 encodes a transcriptional repressor that binds DNA at N-box motifs and is involved in nervous system development and cell differentiation. The gene shows very low constraint against loss-of-function variants (pLI 0.00001, LOEUF 1.95), and currently no definitive disease associations have been established in humans.

Summary from RefSeq, UniProt
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0
Active trials
7
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.95
LOEUF
DN
Mechanism· predicted
Clinical SummaryHES4
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.95LOEUF
pLI 0.000
Z-score -1.22
OE 1.70 (0.821.95)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.47Z-score
OE missense 1.14 (0.971.34)
103 obs / 90.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.70 (0.821.95)
00.351.4
Missense OE1.14 (0.971.34)
00.61.4
Synonymous OE1.27
01.21.6
LoF obs/exp: 6 / 3.5Missense obs/exp: 103 / 90.4Syn Z: -1.37
DN
0.6648th %ile
GOF
0.3887th %ile
LOF
0.49top 25%

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

HES4 · 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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Clinical Literature
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