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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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COMMD3
COMM domain containing 3
Chromosome 10 · 10p12.2
NCBI Gene: 23412Ensembl: ENSG00000148444.17HGNC: HGNC:23332UniProt: Q9UBI1
50PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription FactorTransporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingprotein-containing complexendosome membraneendocytic recyclingneurodegenerative diseasebladder neck obstructionhepatocellular carcinomabreast cancer
✦AI Summary

COMMD3 is a scaffold protein within the commander complex that regulates endosomal recycling of transmembrane cargo proteins 1. It functions as a component of the CCC (COMMD/CCDC22/CCDC93) subcomplex and coordinates endosomal trafficking through interactions with other transport machinery 2. COMMD3 modulates sodium transport in epithelial cells by regulating apical surface expression of ENaC (epithelial sodium channel) subunits 3, affecting Na+ homeostasis and blood pressure regulation. The COMMD3/8 complex serves as a specificity adaptor that recruits GRK6 to chemoattractant receptors, regulating B cell migration and humoral immune responses 4. COMMD3 also modulates melanosomal pH during melanogenesis 5 and participates in α-granule biogenesis in platelets 2. Functionally, COMMD3 regulates lysosomal homeostasis, and loss of COMMD3 impairs lysosomal protein delivery through increased extracellular vesicle release 6. Rare COMMD gene variants are associated with increased Parkinson's disease risk, suggesting COMMD3 modulates neurodegeneration linked to lysosomal dysfunction 6. In hepatocellular carcinoma, elevated COMMD3 expression correlates with poor prognosis and supports cancer cell proliferation 7. COMMD3 is also targetable therapeutically; celastrol selectively inhibits the COMMD3/8 complex, suppressing autoimmune responses in rheumatoid arthritis models 8.

Sources cited
1
COMMD3 functions as a scaffold protein in the commander complex essential for endosomal recycling of transmembrane cargos
PMID: 37172566
2
COMMD3/CCC complex is required for α-granule biogenesis and trafficking of granule proteins through endosomal compartments; COMMD3 deficiency leads to lysosomal degradation of certain granule cargo
PMID: 34905616
3
COMMD3 modulates epithelial sodium transport by reducing ENaC cell surface expression
PMID: 23637203
4
COMMD3/8 complex recruits GRK6 to chemoattractant receptors, regulating B cell migration and humoral immune responses
PMID: 31088898
5
COMMD3 is an endosomal trafficking protein that modulates melanosomal pH during melanogenesis
PMID: 37561850
6
COMMD3 regulates lysosomal homeostasis; loss of COMMD3 increases lysosomal protein release via extracellular vesicles; rare COMMD gene variants associate with increased Parkinson's disease risk
PMID: 40209002
7
High COMMD3 expression is an independent negative prognostic factor in hepatocellular carcinoma and supports HCC cell proliferation
PMID: 34493238
8
Celastrol covalently targets and dissociates the COMMD3/8 complex, suppressing humoral immune responses and autoimmunity in rheumatoid arthritis models
PMID: 37000855
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.49Moderate
bladder neck obstructionOpen Targets
0.16Weak
hepatocellular carcinomaOpen Targets
0.10Weak
breast cancerOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.08Suggestive
Miyoshi myopathyOpen Targets
0.07Suggestive
prostate cancerOpen Targets
0.03Suggestive
androgenetic alopeciaOpen Targets
0.03Suggestive
benign prostatic hyperplasiaOpen Targets
0.03Suggestive
musculoskeletal system diseaseOpen Targets
0.03Suggestive
multiple myelomaOpen Targets
0.02Suggestive
diaphragm diseaseOpen Targets
0.02Suggestive
breast neoplasmOpen Targets
0.02Suggestive
Escherichia coli InfectionsOpen Targets
0.02Suggestive
renal carcinomaOpen Targets
0.02Suggestive
alcohol drinkingOpen Targets
0.02Suggestive
triple-negative breast cancerOpen Targets
0.01Suggestive
ovarian carcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
metastatic prostate cancerOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
VPS29Protein interaction100%CCDC22Protein interaction100%CCDC93Protein interaction100%COMMD1Protein interaction99%DENND10Protein interaction92%VPS26CProtein interaction91%
Tissue Expression6 tissues
Bone Marrow
100%
Heart
93%
Liver
79%
Brain
56%
Lung
50%
Ovary
45%
Gene Interaction Network
Click a node to explore
COMMD3VPS29CCDC22CCDC93COMMD1DENND10VPS26C
PROTEIN STRUCTURE
Preparing viewer…
PDB8P0W · 2.90 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.33LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.98 [0.74–1.33]
RankingsWhere COMMD3 stands among ~20K protein-coding genes
  • #8,790of 20,598
    Most Researched50
  • #13,922of 17,882
    Most Constrained (LOEUF)1.33
Genes detectedCOMMD3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Commander complex regulates lysosomal function and is implicated in Parkinson's disease risk.
PMID: 40209002
Science · 2025
1.00
2
A genome-wide genetic screen uncovers determinants of human pigmentation.
PMID: 37561850
Science · 2023
0.90
3
Celastrol suppresses humoral immune responses and autoimmunity by targeting the COMMD3/8 complex.
PMID: 37000855
Sci Immunol · 2023
0.80
4
Chemoattractant receptor signaling in humoral immunity.
PMID: 38573198
Int Immunol · 2024
0.70
5
Syntaxin 12 and COMMD3 are new factors that function with VPS33B in the biogenesis of platelet α-granules.
PMID: 34905616
Blood · 2022
0.60