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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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GPRASP1
G protein-coupled receptor associated sorting protein 1
Chromosome X · Xq22.1
NCBI Gene: 9737Ensembl: ENSG00000198932.14HGNC: HGNC:24834UniProt: Q5JY77
42PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cytosolprotein bindingendosome to lysosome transportG protein-coupled receptor catabolic processneurodegenerative diseaseIntellectual disabilitybreast cancerbenign prostatic hyperplasia
✦AI Summary

GPRASP1 is a critical regulator of G-protein coupled receptor (GPCR) trafficking and degradation that modulates cellular signaling through lysosomal sorting mechanisms. The protein primarily functions by promoting GPCR degradation through its interaction with BECN2 and the endosomal sorting machinery 1. GPRASP1 facilitates K63-linked ubiquitination of target GPCRs like GPR4 and CXCR4, enhancing their binding to RABGEF1 to activate RAB5 for endosomal conversion and subsequent packaging into multivesicular bodies for lysosomal degradation 2. Loss of GPRASP1 function has significant disease implications across multiple systems. In vascular biology, GPRASP1 variants cause arteriovenous malformations by preventing GPR4 degradation, leading to aberrant GPR4/cAMP/MAPK signaling and endothelial dysfunction 2. In hematopoiesis, GPRASP1 deficiency improves hematopoietic stem cell transplantation outcomes by stabilizing CXCR4, enhancing cell survival and niche retention 3, but paradoxically increases lymphoproliferative disease risk through disrupted B-cell differentiation and excessive somatic hypermutation 4. Clinically, GPRASP1 shows promise as a biomarker in cancers, where reduced expression correlates with better prognosis and enhanced immune infiltration in head and neck and pancreatic cancers 56. Additionally, GPRASP1 deletion prevents adverse effects of chrX β2-adrenoceptor stimulation, suggesting therapeutic potential in respiratory diseases 7.

Sources cited
1
GPRASP1 promotes GPCR degradation through interaction with BECN2 and endosomal sorting machinery
PMID: 37409929
2
GPRASP1 facilitates K63-linked ubiquitination of GPCRs and prevents arteriovenous malformations by regulating GPR4 degradation
PMID: 37787182
3
GPRASP1 deficiency improves hematopoietic stem cell transplantation by stabilizing CXCR4
PMID: 32027737
4
GPRASP1 deficiency increases lymphoproliferative disease risk through disrupted B-cell differentiation
PMID: 39479518
5
Reduced GPRASP1 expression correlates with better prognosis in head and neck cancer
PMID: 36264603
6
GPRASP1 serves as a prognostic biomarker in pancreatic cancer
PMID: 36801507
7
GPRASP1 deletion prevents adverse effects of chronic β2-adrenoceptor stimulation
PMID: 40286598
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.30Weak
Intellectual disabilityOpen Targets
0.16Weak
breast cancerOpen Targets
0.08Suggestive
benign prostatic hyperplasiaOpen Targets
0.08Suggestive
follicular thyroid adenomaOpen Targets
0.07Suggestive
follicular thyroid carcinomaOpen Targets
0.07Suggestive
prostate cancerOpen Targets
0.07Suggestive
cancerOpen Targets
0.06Suggestive
neoplasmOpen Targets
0.05Suggestive
lung cancerOpen Targets
0.05Suggestive
diffuse large B-cell lymphomaOpen Targets
0.03Suggestive
papillary carcinomaOpen Targets
0.03Suggestive
ovarian carcinomaOpen Targets
0.03Suggestive
thyroid cancerOpen Targets
0.03Suggestive
B-cell non-Hodgkins lymphomaOpen Targets
0.03Suggestive
undifferentiated carcinomaOpen Targets
0.03Suggestive
posterior cortical atrophyOpen Targets
0.03Suggestive
glioblastoma multiformeOpen Targets
0.02Suggestive
breast carcinomaOpen Targets
0.02Suggestive
bacteriemiaOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
OPRD1Protein interaction84%OPRM1Protein interaction84%ADRB2Protein interaction76%HMGXB4Shared pathway50%PLEKHF2Shared pathway50%DENND3Shared pathway33%
Tissue Expression6 tissues
Ovary
100%
Brain
53%
Bone Marrow
32%
Heart
26%
Lung
7%
Liver
6%
Gene Interaction Network
Click a node to explore
GPRASP1OPRD1OPRM1ADRB2HMGXB4PLEKHF2DENND3
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q5JY77
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.56Moderately Constrained
pLIⓘ
0.29Tolerant
Observed/Expected LoF0.41 [0.30–0.56]
RankingsWhere GPRASP1 stands among ~20K protein-coding genes
  • #9,880of 20,598
    Most Researched42
  • #3,653of 17,882
    Most Constrained (LOEUF)0.56 · top quartile
Genes detectedGPRASP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
GPRASP1 loss-of-function links to arteriovenous malformations by endothelial activating GPR4 signals.
PMID: 37787182
Brain · 2024
1.00
2
The potent BECN2-ATG14 coiled-coil interaction is selectively critical for endolysosomal degradation of GPRASP1/GASP1-associated GPCRs.
PMID: 37409929
Autophagy · 2023
0.90
3
GPRASP1 is a candidate anti-oncogene and correlates with immune microenvironment and immunotherapeutic efficiency in head and neck cancer.
PMID: 36264603
J Oral Pathol Med · 2023
0.80
4
GPRASP protein deficiency triggers lymphoproliferative disease by affecting B-cell differentiation.
PMID: 39479518
Hemasphere · 2024
0.70
5
GPRASP1 deletion in mice abrogates adverse side effects associated with chronic stimulation of Beta2-adrenoceptor.
PMID: 40286598
Biomed Pharmacother · 2025
0.60