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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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RGP1
RGP1 partner of RAB6A GEF complex
Chromosome 9 · 9p13.3
NCBI Gene: 9827Ensembl: ENSG00000107185.11HGNC: HGNC:21965UniProt: A8K0K1
25PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein-containing complexprotein bindingsmall GTPase bindingretrograde transport, endosome to Golgihereditary spastic paraplegianeurodegenerative diseaseSpastic paraplegiagenetic disorder
✦AI Summary

RGP1 is a component of the RIC1-RGP1 guanine nucleotide exchange factor (GEF) complex that activates the RAB6A GTPase by catalyzing GDP-to-GTP exchange 1. This complex contains an evolutionarily conserved RabGEF domain and an arrestin fold that interact with RAB6A's nucleotide-binding and hypervariable domains, respectively 2. The RIC1-RGP1-RAB6A pathway is essential for retrograde transport of mannose-6-phosphate receptors from endosomes to the Golgi compartment, with RGP1 loss causing destabilization of RAB6A and blocking this trafficking 1. RGP1 also participates in STING trafficking regulation, where its depletion delays STING Golgi exit and enhances innate signaling 3. Structurally, RGP1 belongs to the arrestin-fold protein clan with primarily cytosolic localization 4. Notably, RGP1 was identified as a longevity gene with depleted loss-of-function mutations in centenarians, showing consistent causal effects on aging-related traits and altered expression patterns during aging 5. These findings suggest RGP1's protective role extends beyond membrane trafficking to influence healthy aging and longevity.

Sources cited
1
RIC1-RGP1 complex is a GEF for RAB6A, catalyzes nucleotide exchange, and regulates mannose-6-phosphate receptor retrograde transport
PMID: 23091056
2
Structural basis for RAB6 activation showing RabGEF domain and arrestin fold interactions
PMID: 39632878
3
RGP1 loss increases STING signaling by delaying Golgi exit
PMID: 39657680
4
RGP1 is an arrestin-fold protein with membrane trafficking roles
PMID: 23764049
5
RGP1 identified as longevity gene with depleted loss-of-function mutations in centenarians
PMID: 39424787
Disease Associationsⓘ20
hereditary spastic paraplegiaOpen Targets
0.30Weak
neurodegenerative diseaseOpen Targets
0.22Weak
Spastic paraplegiaOpen Targets
0.15Weak
genetic disorderOpen Targets
0.12Weak
type 2 diabetes mellitusOpen Targets
0.11Weak
atrial fibrillationOpen Targets
0.06Suggestive
restless legs syndromeOpen Targets
0.05Suggestive
migraine disorderOpen Targets
0.04Suggestive
Herpes simplex infectionOpen Targets
0.03Suggestive
hypoparathyroidism, familial isolated, 2Open Targets
0.03Suggestive
neoplasmOpen Targets
0.01Suggestive
human papilloma virus infectionOpen Targets
0.01Suggestive
infectionOpen Targets
0.01Suggestive
IGA glomerulonephritisOpen Targets
0.00Suggestive
adenocarcinomaOpen Targets
0.00Suggestive
autoimmune thrombocytopenic purpuraOpen Targets
0.00Suggestive
lung adenocarcinomaOpen Targets
0.00Suggestive
obesityOpen Targets
0.00Suggestive
Cerebral cortical atrophyOpen Targets
0.00Suggestive
deliriumOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
LAMTOR2Protein interaction100%LAMTOR4Protein interaction100%LAMTOR1Protein interaction100%DOCK1Protein interaction90%LAMTOR3Protein interaction90%LAMTOR5Protein interaction90%
Tissue Expression6 tissues
Heart
100%
Brain
93%
Lung
60%
Ovary
50%
Liver
39%
Bone Marrow
29%
Gene Interaction Network
Click a node to explore
RGP1LAMTOR2LAMTOR4LAMTOR1DOCK1LAMTOR3LAMTOR5
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q92546
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.88LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.64 [0.47–0.88]
RankingsWhere RGP1 stands among ~20K protein-coding genes
  • #13,052of 20,598
    Most Researched25
  • #7,823of 17,882
    Most Constrained (LOEUF)0.88
Genes detectedRGP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Depletion of loss-of-function germline mutations in centenarians reveals longevity genes.
PMID: 39424787
Nat Commun · 2024
1.00
2
Structural basis for Rab6 activation by the Ric1-Rgp1 complex.
PMID: 39632878
Nat Commun · 2024
0.90
3
Receptor deorphanization in starfish reveals the evolution of relaxin signaling as a regulator of reproduction.
PMID: 40001176
BMC Biol · 2025
0.80
4
Classification and functional characterization of regulators of intracellular STING trafficking identified by genome-wide optical pooled screening.
PMID: 39657680
Cell Syst · 2024
0.70
5
Integrated microbiome-metabolome-genome axis data of Laiwu and Lulai pigs.
PMID: 37179393
Sci Data · 2023
0.60