HES7

Chr 17AR

hes family bHLH transcription factor 7

Also known as: SCDO4, bHLHb37, hHes7

This gene encodes a transcriptional repressor that functions as a segmentation clock essential for coordinated somite formation and axial skeleton patterning during embryonic development. Mutations cause spondylocostal dysostosis 4, an autosomal recessive disorder characterized by vertebral and rib malformations. The gene shows high constraint against loss-of-function variants (pLI 0.83, LOEUF 0.52), indicating that heterozygous loss-of-function is likely deleterious.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 0.521 OMIM phenotype
Clinical SummaryHES7
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.83) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.52LOEUF
pLI 0.827
Z-score 2.23
OE 0.00 (0.000.52)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.76Z-score
OE missense 0.79 (0.660.95)
79 obs / 100.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.52)
00.351.4
Missense OE0.79 (0.660.95)
00.61.4
Synonymous OE0.69
01.21.6
LoF obs/exp: 0 / 5.8Missense obs/exp: 79 / 100.4Syn Z: 1.67

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

HES7 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC