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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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GOPC
golgi associated PDZ and coiled-coil motif containing
Chromosome 6 · 6q22.1
NCBI Gene: 57120Ensembl: ENSG00000047932.15HGNC: HGNC:17643UniProt: Q9HD26
137PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
negative regulation of protein localization to cell surfaceGolgi apparatusmembranetrans-Golgi network transport vesiclediabetes mellitusnon-small cell lung carcinomahepatocellular carcinomabile duct adenocarcinoma
✦AI Summary

GOPC (Golgi-associated PDZ and coiled-coil motif containing) is a Golgi-resident trafficking protein that regulates intracellular protein localization and degradation 123. GOPC functions as a molecular platform for controlling plasma membrane expression of multiple proteins, including ion channels and receptors, by modulating their trafficking through the trans-Golgi network and endosomal-lysosomal pathways 4. The protein plays a critical role in insulin secretion by regulating SNAP25 plasma membrane localization at the trans-Golgi network 4. GOPC is also implicated in autophagy-related degradation pathways, particularly through ubiquitin-mediated lysosomal degradation of substrates like CFTR 5. Clinically, GOPC dysfunction is associated with disease pathogenesis in multiple contexts: oncogenic GOPC-ROS1 fusion mutations drive lymphatic malformation formation and mediate tyrosine kinase signaling 67, and GOPC is targeted for degradation by HSV-1 pUL56, enabling viral evasion of immune responses through altered cell-surface receptor expression 8. Additionally, altered GOPC expression correlates with preeclampsia pathology 9, suggesting broader roles in vascular and metabolic diseases.

Sources cited
1
GOPC plays a role in intracellular protein trafficking and degradation; overexpression results in CFTR intracellular retention and lysosomal degradation
PMID: 11707463
2
GOPC regulates intracellular protein trafficking and degradation; overexpression leads to CFTR lysosomal degradation
PMID: 14570915
3
GOPC regulates intracellular protein trafficking and degradation; involved in ADR1B receptor intracellular trafficking
PMID: 15358775
4
GOPC together with MARCHF2 mediates ubiquitination and lysosomal degradation of CFTR
PMID: 23818989
5
GOPC is required for insulin secretion by regulating SNAP25 plasma membrane localization at the trans-Golgi network through interaction with ARFRP1
PMID: 33359402
6
Oncogenic GOPC-ROS1 fusion promotes lymphatic malformation formation through altered tyrosine kinase signaling and impaired tight junction function
PMID: 40819798
7
GOPC::ROS1 fusion is a rare gene alteration identified in pediatric low-grade glioma by RNA-Seq
PMID: 37075810
8
HSV-1 pUL56 targets GOPC for ubiquitination and proteasomal degradation; GOPC regulates cell-surface expression of TLR2
PMID: 33027661
9
GOPC is differentially methylated and upregulated in preeclampsia placental tissues
PMID: 36660680
Disease Associationsⓘ20
diabetes mellitusOpen Targets
0.40Moderate
non-small cell lung carcinomaOpen Targets
0.37Weak
hepatocellular carcinomaOpen Targets
0.37Weak
bile duct adenocarcinomaOpen Targets
0.37Weak
brain astrocytomaOpen Targets
0.37Weak
type 2 diabetes mellitusOpen Targets
0.32Weak
hypertensionOpen Targets
0.29Weak
neurodegenerative diseaseOpen Targets
0.28Weak
lung adenocarcinomaOpen Targets
0.28Weak
Borderline Ovarian Serous TumorOpen Targets
0.28Weak
brain glioblastomaOpen Targets
0.28Weak
small cell lung carcinomaOpen Targets
0.19Weak
esophageal adenocarcinomaOpen Targets
0.19Weak
head and neck squamous cell carcinomaOpen Targets
0.19Weak
acute lymphoblastic leukemiaOpen Targets
0.19Weak
skin basal cell carcinomaOpen Targets
0.19Weak
bladder transitional cell carcinomaOpen Targets
0.19Weak
cervical squamous cell carcinomaOpen Targets
0.19Weak
gastric adenocarcinomaOpen Targets
0.19Weak
squamous cell lung carcinomaOpen Targets
0.19Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CFTRProtein interaction99%NHERF2Protein interaction99%NHERF1Protein interaction99%RHOQProtein interaction99%STX6Protein interaction97%CD46Protein interaction89%
Tissue Expression6 tissues
Heart
100%
Brain
80%
Ovary
72%
Liver
49%
Lung
41%
Bone Marrow
34%
Gene Interaction Network
Click a node to explore
GOPCCFTRNHERF2NHERF1RHOQSTX6CD46
PROTEIN STRUCTURE
Preparing viewer…
PDB4JOK · 1.09 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.81LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.57 [0.41–0.81]
RankingsWhere GOPC stands among ~20K protein-coding genes
  • #3,375of 20,598
    Most Researched137 · top quartile
  • #6,839of 17,882
    Most Constrained (LOEUF)0.81
Genes detectedGOPC
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Impaired autophagy and APP processing in Alzheimer's disease: The potential role of Beclin 1 interactome.
PMID: 23827971
Prog Neurobiol · 2013
1.00
2
Oncogenic GOPC-ROS1 fusion promotes the formation of lymphatic malformations by impairing tight junction and its crizotinib resistance through repressing ferroptosis.
PMID: 40819798
Biochem Pharmacol · 2025
0.90
3
LOGGIC Core BioClinical Data Bank: Added clinical value of RNA-Seq in an international molecular diagnostic registry for pediatric low-grade glioma patients.
PMID: 37075810
Neuro Oncol · 2023
0.80
4
Defining hard-to-reach populations for vaccination.
PMID: 31400910
Vaccine · 2019
0.70
5
The ARFRP1-dependent Golgi scaffolding protein GOPC is required for insulin secretion from pancreatic β-cells.
PMID: 33359402
Mol Metab · 2021
0.60