HSPB8

Chr 12

heat shock protein family B (small) member 8

Also known as: CMT2L, DHMN2, E2IG1, H11, HMN2, HMN2A, HMND2, HSP22

The protein encoded by this gene belongs to the superfamily of small heat-shock proteins containing a conservative alpha-crystallin domain at the C-terminal part of the molecule. The expression of this gene in induced by estrogen in estrogen receptor-positive breast cancer cells, and this protein also functions as a chaperone in association with Bag3, a stimulator of macroautophagy. Thus, this gene appears to be involved in regulation of cell proliferation, apoptosis, and carcinogenesis, and mutations in this gene have been associated with different neuromuscular diseases, including Charcot-Marie-Tooth disease. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtNeuronopathy, distal hereditary motor, autosomal dominant 2
UniProtCharcot-Marie-Tooth disease, axonal, type 2L
UniProtMyopathy, myofibrillar, 13, with rimmed vacuoles

Clinical highlights

Gene-disease validity (ClinGen)
neuronopathy, distal hereditary motor, autosomal dominant · ADDefinitivesufficient evidence for diagnostic panels
1
Active trials
39
Pubs (1 yr)
P/LP submissions
P/LP missense
1.14
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — HSPB8
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.14LOEUF
pLI 0.043
Z-score 1.35
OE 0.44 (0.201.14)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.03Z-score
OE missense 0.99 (0.851.16)
110 obs / 110.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.44 (0.201.14)
00.351.4
Missense OE?0.99 (0.851.16)
00.61.4
Synonymous OE?1.09
01.21.6
LoF obs/exp: 3 / 6.8Missense obs/exp: 110 / 110.8Syn Z: -0.46

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

HSPB8 · protein map & ClinVar variants

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