HSP90B1

Chr 12

heat shock protein 90 beta family member 1

Also known as: ECGP, GP96, GRP94, HEL-S-125m, HEL35, TRA1

The protein is an ATP-dependent molecular chaperone localized to the endoplasmic reticulum that regulates protein folding and transport, including folding of Wnt pathway components and Toll-like receptors. Mutations cause autosomal recessive neurodevelopmental disorders with epilepsy and intellectual disability. This gene is highly constrained against loss-of-function variants in the general population.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.27
Clinical SummaryHSP90B1
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.27LOEUF
pLI 0.997
Z-score 5.29
OE 0.14 (0.070.27)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.12Z-score
OE missense 0.71 (0.650.78)
307 obs / 431.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.14 (0.070.27)
00.351.4
Missense OE0.71 (0.650.78)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 6 / 43.7Missense obs/exp: 307 / 431.1Syn Z: 0.20

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

HSP90B1 · 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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