CHMP2B

Chr 3

charged multivesicular body protein 2B

Also known as: ALS17, CHMP2.5, DMT1, FTDALS7, VPS2-2, VPS2B

This gene encodes a component of the heteromeric ESCRT-III complex (Endosomal Sorting Complex Required for Transport III) that functions in the recycling or degradation of cell surface receptors. ESCRT-III functions in the concentration and invagination of ubiquitinated endosomal cargos into intralumenal vesicles. The protein encoded by this gene is found as a monomer in the cytosol or as an oligomer in ESCRT-III complexes on endosomal membranes. It is expressed in neurons of all major regions of the brain. Mutations in this gene result in one form of familial frontotemporal lobar degeneration. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtFrontotemporal dementia and/or amyotrophic lateral sclerosis 7

Clinical highlights

Gene-disease validity (ClinGen)
frontotemporal dementia and/or amyotrophic lateral sclerosis 7 · ADDefinitivesufficient evidence for diagnostic panels
0
Active trials
29
Pubs (1 yr)
P/LP submissions
P/LP missense
1.22
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — CHMP2B
Authoritative clinical overview · Recommended first read
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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?
1.22LOEUF
pLI 0.000
Z-score 0.98
OE 0.72 (0.441.22)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.34Z-score
OE missense 0.91 (0.781.07)
103 obs / 113.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.72 (0.441.22)
00.351.4
Missense OE?0.91 (0.781.07)
00.61.4
Synonymous OE?0.97
01.21.6
LoF obs/exp: 10 / 14.0Missense obs/exp: 103 / 113.2Syn Z: 0.14

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CHMP2B · 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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