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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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GOLPH3L
golgi phosphoprotein 3 like
Chromosome 1 · 1q21.3
NCBI Gene: 55204Ensembl: ENSG00000143457.12HGNC: HGNC:24882UniProt: Q9H4A5
36PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingphosphatidylinositol-4-phosphate bindingcargo adaptor activityGolgi organizationinflammatory bowel diseasecutaneous melanomaCrohn's diseasepericarditis
✦AI Summary

GOLPH3L (golgi phosphoprotein 3 like) is a phosphatidylinositol-4-phosphate (PI4P) binding protein that localizes to the Golgi apparatus and plays crucial roles in Golgi organization and protein trafficking 1. Unlike its paralogue GOLPH3, GOLPH3L functions as an antagonist to GOLPH3/MYO18A signaling, producing opposite effects on Golgi morphology despite similar PI4P binding capabilities 1. GOLPH3L exhibits tissue-specific expression patterns, being particularly abundant in secretory tissues, contrasting with the ubiquitous expression of GOLPH3 1. Together with GOLPH3, GOLPH3L serves as a broad-spectrum COPI adaptor that maintains Golgi enzyme localization and ensures proper glycosylation pathway function 2. The protein is essential for maintaining the cis-Golgi localization of the LYSET-GNPT complex, which is critical for mannose 6-phosphate tagging machinery and lysosomal homeostasis 3. GOLPH3L has significant clinical relevance, as it is overexpressed in various cancers including rhabdomyosarcoma and breast cancer, where it promotes tumor cell proliferation and metabolic reprogramming 45. In glioblastoma, GOLPH3L contributes to radiotherapy resistance by suppressing STING-mediated immune responses, making it a potential therapeutic target 6.

Sources cited
1
GOLPH3L binds PI4P, localizes to Golgi, antagonizes GOLPH3/MYO18A, and shows tissue-specific expression
PMID: 23345592
2
GOLPH3L acts as a broad-spectrum COPI adaptor for Golgi enzyme retention and glycosylation pathways
PMID: 34473204
3
GOLPH3L maintains cis-Golgi localization of LYSET-GNPT complex and M6P-tagging machinery
PMID: 39587297
4
GOLPH3L is overexpressed in rhabdomyosarcoma and promotes tumor cell proliferation
PMID: 21822541
5
GOLPH3L is overexpressed in breast cancer and promotes metabolic reprogramming
PMID: 33509226
6
GOLPH3L contributes to glioblastoma radiotherapy resistance by suppressing STING-mediated immune responses
PMID: 40043140
Disease Associationsⓘ20
inflammatory bowel diseaseOpen Targets
0.17Weak
cutaneous melanomaOpen Targets
0.16Weak
Crohn's diseaseOpen Targets
0.14Weak
pericarditisOpen Targets
0.14Weak
atrial fibrillationOpen Targets
0.11Weak
skin carcinomaOpen Targets
0.08Suggestive
breast cancerOpen Targets
0.08Suggestive
ovarian cancerOpen Targets
0.08Suggestive
skin neoplasmOpen Targets
0.07Suggestive
cervical cancerOpen Targets
0.06Suggestive
Alzheimer diseaseOpen Targets
0.05Suggestive
achalasia-alacrima syndromeOpen Targets
0.04Suggestive
neoplasmOpen Targets
0.04Suggestive
coronary artery diseaseOpen Targets
0.04Suggestive
skin cancerOpen Targets
0.04Suggestive
type 2 diabetes mellitusOpen Targets
0.03Suggestive
diabetes mellitusOpen Targets
0.03Suggestive
prostate carcinomaOpen Targets
0.03Suggestive
kidney failureOpen Targets
0.03Suggestive
cancerOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PCSK9Protein interaction86%MYO18AProtein interaction55%BLZF1Shared pathway50%COG4Shared pathway40%GOLPH3Shared pathway29%GOLGA8RShared pathway25%
Tissue Expression6 tissues
Ovary
100%
Brain
99%
Heart
99%
Lung
75%
Liver
67%
Bone Marrow
45%
Gene Interaction Network
Click a node to explore
GOLPH3LPCSK9MYO18ABLZF1COG4GOLPH3GOLGA8R
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9H4A5
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.51LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.94 [0.60–1.51]
RankingsWhere GOLPH3L stands among ~20K protein-coding genes
  • #10,783of 20,598
    Most Researched36
  • #15,240of 17,882
    Most Constrained (LOEUF)1.51
Genes detectedGOLPH3L
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
GOLPH3 and GOLPH3L maintain Golgi localization of LYSET and a functional mannose 6-phosphate transport pathway.
PMID: 39587297
EMBO J · 2024
1.00
2
Role of GOLPH3 and GOLPH3L in the proliferation of human rhabdomyosarcoma.
PMID: 21822541
Oncol Rep · 2011
0.90
3
Targeting GOLPH3L improves glioblastoma radiotherapy by regulating STING-NLRP3-mediated tumor immune microenvironment reprogramming.
PMID: 40043140
Sci Transl Med · 2025
0.80
4
Ythdc1-p300-Klf5 Complex-Mediated Golgi Dysfunction Promotes Aortic Aneurysm.
PMID: 41317401
Adv Sci (Weinh) · 2026
0.70
5
GOLPH3 and GOLPH3L are broad-spectrum COPI adaptors for sorting into intra-Golgi transport vesicles.
PMID: 34473204
J Cell Biol · 2021
0.60