CHMP7

Chr 8

charged multivesicular body protein 7

The CHMP7 protein is an ESCRT-III-like component required for nuclear envelope sealing and mitotic spindle disassembly during cell division, and also functions in endosomal sorting pathways. Mutations cause autosomal recessive microcephaly, seizures, and developmental delay. This gene is highly constrained against loss-of-function variants, indicating that complete protein loss is likely incompatible with normal development.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
10
Pubs (1 yr)
81
P/LP submissions
0%
P/LP missense
0.38
LOEUF
Mechanism
Clinical SummaryCHMP7
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.86) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
81 unique Pathogenic / Likely Pathogenic· 61 VUS of 163 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.38LOEUF
pLI 0.863
Z-score 3.80
OE 0.17 (0.080.38)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.50Z-score
OE missense 0.74 (0.650.83)
191 obs / 259.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.17 (0.080.38)
00.351.4
Missense OE0.74 (0.650.83)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 4 / 24.1Missense obs/exp: 191 / 259.1Syn Z: 0.08

ClinVar Variant Classifications

163 submitted variants in ClinVar

Classification Summary

Pathogenic77
Likely Pathogenic4
VUS61
Likely Benign1
Benign2
77
Pathogenic
4
Likely Pathogenic
61
VUS
1
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
77
0
77
Likely Pathogenic
0
0
4
0
4
VUS
0
57
4
0
61
Likely Benign
0
0
1
0
1
Benign
0
0
0
2
2
Total057862145

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

CHMP7 · 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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