COMMD9

Chr 11

COMM domain containing 9

Also known as: C11orf55, HSPC166, LINC00610

COMMD9 encodes a scaffold protein that is essential for endosomal recycling of transmembrane cargos as part of the commander complex, and modulates sodium transport in epithelial cells by regulating amiloride-sensitive sodium channel expression at the cell surface. Mutations in COMMD9 cause autosomal recessive neurodevelopmental disorder with microcephaly, seizures, and brain atrophy. The disorder presents in infancy with developmental delays, seizures, and progressive brain volume loss affecting multiple neurological systems.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
3
Pubs (1 yr)
15
P/LP submissions
0%
P/LP missense
0.68
LOEUF
Mechanism
Clinical SummaryCOMMD9
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.22) despite low pLI — interpret in context.
📋
ClinVar Variants
15 unique Pathogenic / Likely Pathogenic· 28 VUS of 55 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.68LOEUF
pLI 0.394
Z-score 2.21
OE 0.22 (0.090.68)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.48Z-score
OE missense 0.87 (0.741.03)
97 obs / 111.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.22 (0.090.68)
00.351.4
Missense OE0.87 (0.741.03)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 2 / 9.3Missense obs/exp: 97 / 111.2Syn Z: -0.24

ClinVar Variant Classifications

55 submitted variants in ClinVar

Classification Summary

Pathogenic14
Likely Pathogenic1
VUS28
Likely Benign1
Benign2
14
Pathogenic
1
Likely Pathogenic
28
VUS
1
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
14
0
14
Likely Pathogenic
0
0
1
0
1
VUS
0
20
8
0
28
Likely Benign
0
1
0
0
1
Benign
0
0
1
1
2
Total02124146

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

View in ClinVar →

Protein Context — Lollipop Plot

COMMD9 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC