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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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COP1
COP1 E3 ubiquitin ligase
Chromosome 1 · 1q25.1-q25.2
NCBI Gene: 64326Ensembl: ENSG00000143207.22HGNC: HGNC:17440UniProt: Q8NHY2
121PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
negative regulation of fatty acid biosynthetic processprotein bindingubiquitin protein ligase activityubiquitin-dependent protein catabolic processAbnormality of the skeletal systemmajor depressive disorderirritable bowel syndromeosteonecrosis
✦AI Summary

COP1 is an E3 ubiquitin ligase that mediates proteasomal degradation of multiple substrate proteins, functioning as a RING domain subunit within larger E3 ubiquitin ligase complexes 1. Primary substrates include p53, c-Jun, c/EBPβ, c/EBPδ, FOXO1, and 14-3-3σ, whose degradation suppresses tumor suppression and apoptosis 234. In cancer immunotherapy, COP1 deletion in tumor cells stabilizes c/EBPδ, reducing macrophage chemoattractant secretion and enhancing anti-tumor immunity with improved checkpoint blockade response in triple-negative breast cancer 4. In neuroinflammation, COP1 suppresses pathogenic microglial activation by degrading c/EBPβ; COP1 deficiency accelerates tau-mediated neurodegeneration through complement-dependent mechanisms 3. COP1 exhibits context-dependent roles: in glucose-driven tumorigenesis, COP1-mediated p53 degradation promotes glycolysis and cancer cell proliferation, while disrupting COP1-p53 interaction reverses hyperglycemia-induced tumorigenesis 2. In hepatocellular carcinoma, TRIB3-recruited COP1 degrades FOXO1, promoting MET-driven tumor development 5. These dual tumor-promoting and immune-suppressive functions identify COP1 as a potential therapeutic target for cancer immunotherapy and metabolic disease management.

Sources cited
1
COP1 deletion in cancer cells decreases macrophage infiltration and enhances ICB response; COP1-Trib2-C/ebpδ axis regulates chemokine secretion
PMID: 34582788
2
COP1 degrades c/EBPβ to suppress neuroinflammation; COP1 deficiency accelerates tau-mediated neurodegeneration
PMID: 32795415
3
Glucose-induced CRL4COP1 targets p53 for degradation to derepress glycolytic enzymes and promote cancer cell proliferation
PMID: 37390815
4
TRIB3 recruits COP1 to mediate FOXO1 ubiquitination and degradation in MET-driven hepatocellular carcinoma
PMID: 40211872
5
COP1 is an E3 ubiquitin ligase with multiple substrates including p53, c-Jun, c/EBPα, β-catenin, STAT3, MTA1, and 14-3-3σ; plays tumor-suppressive and oncogenic roles in malignancies
PMID: 32027978
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.40Weak
major depressive disorderOpen Targets
0.39Weak
irritable bowel syndromeOpen Targets
0.37Weak
osteonecrosisOpen Targets
0.32Weak
hypertensionOpen Targets
0.32Weak
lagophthalmosOpen Targets
0.29Weak
obesityOpen Targets
0.28Weak
supraventricular ectopyOpen Targets
0.27Weak
Abruptio PlacentaeOpen Targets
0.27Weak
gestational diabetesOpen Targets
0.26Weak
COVID-19Open Targets
0.26Weak
major depressive episodeOpen Targets
0.25Weak
schizophreniaOpen Targets
0.25Weak
pyelonephritisOpen Targets
0.23Weak
Limb painOpen Targets
0.23Weak
bipolar disorderOpen Targets
0.21Weak
Tietze syndromeOpen Targets
0.21Weak
Abdominal painOpen Targets
0.20Weak
HeadacheOpen Targets
0.20Weak
mixed connective tissue diseaseOpen Targets
0.19Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
GLMNProtein interaction100%FOSProtein interaction100%CDKN1BProtein interaction99%ERCC8Protein interaction99%DDB1Protein interaction99%DDB2Protein interaction99%
Tissue Expression6 tissues
Brain
100%
Lung
100%
Bone Marrow
87%
Liver
84%
Heart
73%
Ovary
56%
Gene Interaction Network
Click a node to explore
COP1GLMNFOSCDKN1BERCC8DDB1DDB2
PROTEIN STRUCTURE
Preparing viewer…
PDB5HQG · 2.00 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.56Moderately Constrained
pLIⓘ
0.92Intolerant
Observed/Expected LoF0.30 [0.17–0.56]
RankingsWhere COP1 stands among ~20K protein-coding genes
  • #3,877of 20,598
    Most Researched121 · top quartile
  • #3,604of 17,882
    Most Constrained (LOEUF)0.56 · top quartile
Genes detectedCOP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
In vivo CRISPR screens identify the E3 ligase Cop1 as a modulator of macrophage infiltration and cancer immunotherapy target.
PMID: 34582788
Cell · 2021
1.00
2
Ubiquitin Ligase COP1 Suppresses Neuroinflammation by Degrading c/EBPβ in Microglia.
PMID: 32795415
Cell · 2020
0.90
3
ER-to-Golgi protein delivery through an interwoven, tubular network extending from ER.
PMID: 33852913
Cell · 2021
0.80
4
Glucose-induced CRL4
PMID: 37390815
Mol Cell · 2023
0.70
5
Retraction.
PMID: 33988266
J Cell Biochem · 2021
0.60