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25 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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COPS3
COP9 signalosome subunit 3
Chromosome 17 · 17p11.2
NCBI Gene: 8533Ensembl: ENSG00000141030.14HGNC: HGNC:2239UniProt: B4DN01
181PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cytoplasmcell-cell junctionperinuclear region of cytoplasmCOP9 signalosomeneurodegenerative diseaseosteosarcomalung cancerneoplasm
✦AI Summary

COPS3 (COP9 signalosome subunit 3) is a critical component of the COP9 signalosome complex that regulates ubiquitin-dependent protein degradation through deneddylation of cullin-containing E3 ligases. Beyond this canonical role, COPS3 functions as a prominent oncogene across multiple cancer types. In osteosarcoma, COPS3 amplification occurs in 31% of tumors and correlates with large tumor size and increased metastatic risk 1. COPS3 promotes metastasis through multiple mechanisms: it directly interacts with Raf-1 to activate MEK/ERK signaling and regulate epithelial-mesenchymal transition (EMT) 2, and it interacts with Beclin1 to enhance autophagic flux 2. COPS3 also establishes a positive feedback loop with FOXO3 that promotes cytoprotective autophagy, driving cisplatin resistance 3. Additionally, COPS3 regulates anoikis resistance through the PFKFB3-glycolysis pathway 4. In colorectal cancer, COPS3 depletion suppresses cell proliferation, viability, motility, and EMT via MEK/ERK pathway inhibition 5. COPS3's oncogenic activity suggests it represents a promising therapeutic target for chemoresistant and metastatic cancers.

Sources cited
1
COPS3 amplification detected in 31% of osteosarcomas, associated with large tumor size and shorter time to metastasis
PMID: 17366602
2
COPS3 directly interacts with Raf-1 and Beclin1 to promote MEK/ERK signaling, EMT, and autophagic flux in osteosarcoma metastasis
PMID: 29970115
3
COPS3-FOXO3 positive feedback loop regulates autophagy to promote cisplatin resistance in osteosarcoma
PMID: 36451342
4
COPS3 modulates anoikis and metastasis through PFKFB3-mediated glycolysis regulation in osteosarcoma
PMID: 37201626
5
COPS3 depletion suppresses colorectal cancer cell proliferation, viability, motility, and EMT via MEK/ERK pathway inhibition
PMID: 35903293
6
COPS3 expression correlates with metastasis in osteosarcoma; COPS3 silencing inhibits MAPK signaling and cell migration
PMID: 21436869
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.42Moderate
osteosarcomaOpen Targets
0.10Suggestive
lung cancerOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.06Suggestive
type 2 diabetes mellitusOpen Targets
0.02Suggestive
posterior cortical atrophyOpen Targets
0.02Suggestive
COVID-19Open Targets
0.02Suggestive
squamous cell carcinomaOpen Targets
0.02Suggestive
eating disorderOpen Targets
0.02Suggestive
adenocarcinomaOpen Targets
0.02Suggestive
cancerOpen Targets
0.02Suggestive
small cell carcinomaOpen Targets
0.02Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.01Suggestive
head and neck squamous cell carcinomaOpen Targets
0.01Suggestive
colorectal cancerOpen Targets
0.01Suggestive
prostate cancerOpen Targets
0.01Suggestive
Smith-Magenis syndromeOpen Targets
0.01Suggestive
potassium deficiencyOpen Targets
0.01Suggestive
clear cell renal carcinomaOpen Targets
0.01Suggestive
sarcomaOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
UBE2MProtein interaction100%DCAF1Protein interaction100%CRBNProtein interaction100%CUL3Protein interaction100%DDB1Protein interaction100%ELOCProtein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
82%
Heart
55%
Lung
33%
Liver
28%
Ovary
26%
Gene Interaction Network
Click a node to explore
COPS3UBE2MDCAF1CRBNCUL3DDB1ELOC
PROTEIN STRUCTURE
Preparing viewer…
PDB4D10 · 3.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.35Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.20 [0.13–0.35]
RankingsWhere COPS3 stands among ~20K protein-coding genes
  • #2,394of 20,598
    Most Researched181 · top quartile
  • #1,525of 17,882
    Most Constrained (LOEUF)0.35 · top 10%
Genes detectedCOPS3
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
The COPS3-FOXO3 positive feedback loop regulates autophagy to promote cisplatin resistance in osteosarcoma.
PMID: 36451342
Autophagy · 2023
1.00
2
Silencing of the COPS3 gene by siRNA reduces proliferation of lung cancer cells most likely via induction of cell cycle arrest and apoptosis.
PMID: 22631635
Asian Pac J Cancer Prev · 2012
0.90
3
COPS3 inhibition promotes cell proliferation blockage and anoikis via regulating PFKFB3 in osteosarcoma cancer cells.
PMID: 37201626
Eur J Pharmacol · 2023
0.80
4
COPS3 Promotes Proliferation, Invasion, and EMT of Colorectal Cancer Cells by MEK/ERK Pathway.
PMID: 35903293
Dis Markers · 2022
0.70
5
Gut Microbiota and White Matter Integrity: A Two-Sample Mendelian Randomization Analysis.
PMID: 40825663
eNeuro · 2025
0.68