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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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GPR88
G protein-coupled receptor 88
Chromosome 1 · 1p21.2
NCBI Gene: 54112Ensembl: ENSG00000181656.7HGNC: HGNC:4539UniProt: Q9GZN0
13PubMed Papers
21Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Receptor
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
beta2-adrenergic receptor activityciliumciliary membraneG protein-coupled receptor signaling pathwaychorea, childhood-onset, with psychomotor retardationbrain injuryopen-angle glaucomaexternal ear disease
✦AI Summary

GPR88 is an orphan G protein-coupled receptor predominantly expressed in the brain, particularly in the striatum, that plays crucial roles in cognitive function, motor control, and emotional processing 1. The receptor couples to Gi/o signaling pathways and demonstrates constitutive activity 2. GPR88 functions as a negative regulator of other GPCR signaling, inhibiting both μ-opioid receptor and kappa-opioid receptor (KOR) responses through direct protein-protein interactions 3. This inhibitory function appears particularly important in dopamine D1-receptor-containing neurons of the basal ganglia direct pathway 3. Disease relevance includes associations with childhood-onset chorea with psychomotor retardation, where GPR88 mutations are among the genetic causes 4. The receptor is also implicated in various neuropsychiatric disorders including schizophrenia, Parkinson's disease, anxiety, and addiction 15. Additionally, GPR88 has emerged as a potential biomarker in metabolic associated steatotic liver disease 6. Despite its therapeutic potential as a neurotherapeutic target, effective synthetic ligands remain under development, with recent compounds like BI-9508 showing promise as research tools 7. The receptor's brain-specific expression profile and involvement in multiple CNS disorders make it an attractive target for novel therapeutic development.

Sources cited
1
GPR88 is brain-specific with robust striatal expression and regulates cognition, mood, movement control, and reward-based learning
PMID: 30540906
2
GPR88 demonstrates constitutive Gi/o signaling activity
PMID: 33784795
3
GPR88 interacts with and inhibits kappa-opioid receptor signaling, particularly in D1-receptor neurons
PMID: 39613254
4
GPR88 mutations cause childhood-onset chorea with psychomotor retardation
PMID: 32776155
5
GPR88 is implicated in schizophrenia and other neuropsychiatric disorders
PMID: 37605621
6
GPR88 serves as a potential biomarker in metabolic associated steatotic liver disease
PMID: 40275274
7
BI-9508 represents a brain-penetrant GPR88 agonist tool compound
PMID: 38949964
Disease Associationsⓘ21
chorea, childhood-onset, with psychomotor retardationOpen Targets
0.50Moderate
brain injuryOpen Targets
0.15Weak
open-angle glaucomaOpen Targets
0.15Weak
external ear diseaseOpen Targets
0.15Weak
alcohol drinkingOpen Targets
0.13Weak
coronary artery bypassOpen Targets
0.12Weak
AnxietyOpen Targets
0.06Suggestive
glaucomaOpen Targets
0.05Suggestive
autismOpen Targets
0.05Suggestive
behavioral variant of frontotemporal dementiaOpen Targets
0.05Suggestive
15q11q13 microduplication syndromeOpen Targets
0.04Suggestive
Pick diseaseOpen Targets
0.04Suggestive
autism spectrum disorderOpen Targets
0.04Suggestive
childhood disintegrative disorderOpen Targets
0.04Suggestive
Parkinson diseaseOpen Targets
0.04Suggestive
amyotrophic lateral sclerosisOpen Targets
0.03Suggestive
frontotemporal dementiaOpen Targets
0.03Suggestive
familial amyotrophic lateral sclerosisOpen Targets
0.03Suggestive
N-acetylaspartate deficiencyOpen Targets
0.03Suggestive
intellectual disability, autosomal dominant 50Open Targets
0.03Suggestive
Chorea, childhood-onset, with psychomotor retardationUniProt
Pathogenic Variants1
NM_022049.3(GPR88):c.873C>A (p.Cys291Ter)Pathogenic
Chorea, childhood-onset, with psychomotor retardation
☆☆☆☆2024→ Residue 291
View on ClinVar ↗
Related Genes
GNB1Protein interaction92%GNAI1Protein interaction92%GNG2Protein interaction83%GPR6Protein interaction65%GPR52Protein interaction62%RRHShared pathway50%
Tissue Expression6 tissues
Liver
100%
Ovary
43%
Brain
42%
Heart
13%
Bone Marrow
8%
Lung
1%
Gene Interaction Network
Click a node to explore
GPR88GNB1GNAI1GNG2GPR6GPR52RRH
PROTEIN STRUCTURE
Preparing viewer…
PDB7EJX · 2.40 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.77LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.23 [0.85–1.77]
RankingsWhere GPR88 stands among ~20K protein-coding genes
  • #16,178of 20,598
    Most Researched13
  • #5,452of 5,498
    Most Pathogenic Variants1
  • #16,429of 17,882
    Most Constrained (LOEUF)1.77
Genes detectedGPR88
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Chorea in children: etiology, diagnostic approach and management.
PMID: 32776155
J Neural Transm (Vienna) · 2020
1.00
2
Identifying disease progression biomarkers in metabolic associated steatotic liver disease (MASLD) through weighted gene co-expression network analysis and machine learning.
PMID: 40275274
J Transl Med · 2025
0.90
3
Orphan Receptor GPR88 as an Emerging Neurotherapeutic Target.
PMID: 30540906
ACS Chem Neurosci · 2019
0.80
4
Discovery of BI-9508, a Brain-Penetrant GPR88-Receptor-Agonist Tool Compound for
PMID: 38949964
J Med Chem · 2024
0.70
5
Orphan G Protein-Coupled Receptor GPR88: Mapping Its Significance in Central Nervous System Disorders.
PMID: 40184024
Mol Neurobiol · 2025
0.60