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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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GPR161
G protein-coupled receptor 161
Chromosome 1 · 1q24.2
NCBI Gene: 23432Ensembl: ENSG00000143147.15HGNC: HGNC:23694UniProt: A0A0A0MQW8
35PubMed Papers
21Diseases
0Drugs
2Pathogenic Variants
FUNCTIONAL ROLE
Receptor
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingnegative regulation of smoothened signaling pathwayG protein-coupled receptor activitynon-motile cilium membranemedulloblastomapituitary stalk interruption syndromeprostatitisneural tube defects, folate-sensitive
✦AI Summary

GPR161 is an orphan G protein-coupled receptor that serves as a critical negative regulator of Hedgehog signaling during neural development 1. Located at the primary cilium, GPR161 increases cAMP levels to activate PKA-dependent phosphorylation of GLI3, promoting its conversion to the repressor form GLI3R and suppressing Hedgehog pathway activity 1. GPR161 is recruited to cilia through interactions with the IFT-A complex and functions as part of a macromolecular signaling complex with PKA holoenzymes 1. Upon Hedgehog activation, GPR161 undergoes phosphorylation by GRK2, recruits β-arrestins, and is exported from cilia via the BBSome and IFT machinery, allowing Hedgehog signaling activation 2. Structurally, GPR161 contains an unusual extracellular loop occupying the ligand pocket and binds sterols that stabilize Gs coupling, though its cognate ligand remains unknown 3. Recent evidence reveals GPR161 functions as a mechanoreceptor at primary cilia, with its helix 8 sensing fluid shear stress to regulate neuronal migration through cAMP/PKA-mediated phosphorylation of NDE1 4. GPR161 dysfunction is associated with medulloblastoma development 5, and targeting GPR161-mediated Hedgehog signaling shows therapeutic potential in inflammatory disease models 5.

Sources cited
1
GPR161 is an orphan GPCR that increases cAMP levels and activates PKA to suppress Hedgehog signaling in primary cilia by promoting GLI3 processing
PMID: 31947770
2
GPR161 structure reveals sterol binding adjacent to transmembrane helices 6-7 that stabilizes Gs coupling, and a PKA-binding site on C-terminus critical for GLI2 repression
PMID: 38326651
3
GPR161 functions as a mechanoreceptor at primary cilia, with mechanosensitive helix 8 sensing fluid shear stress to regulate neuronal migration
PMID: 40737401
4
GPR161 is phosphorylated by GRK2, recruits β-arrestins, and is exported from cilia via BBSome and IFT machinery upon Hedgehog activation
PMID: 40384633
5
GPR161 downregulation activates Hedgehog pathway signaling and is associated with medulloblastoma; GPR161-targeting represents therapeutic strategy
PMID: 37327587
Disease Associationsⓘ21
medulloblastomaOpen Targets
0.58Moderate
pituitary stalk interruption syndromeOpen Targets
0.44Moderate
prostatitisOpen Targets
0.27Weak
neural tube defects, folate-sensitiveOpen Targets
0.07Suggestive
anencephaly 1Open Targets
0.06Suggestive
Isolated anencephaly/exencephalyOpen Targets
0.06Suggestive
Spina bifida - hypospadiasOpen Targets
0.06Suggestive
spina bifida-hypospadias syndromeOpen Targets
0.06Suggestive
tropical spastic paraparesisOpen Targets
0.06Suggestive
early-onset non-syndromic cataractOpen Targets
0.05Suggestive
neoplasmOpen Targets
0.05Suggestive
Cataract-microcornea syndromeOpen Targets
0.05Suggestive
uncombable hair syndrome 2Open Targets
0.05Suggestive
Griscelli diseaseOpen Targets
0.05Suggestive
Familial ocular anterior segment mesenchymal dysgenesisOpen Targets
0.05Suggestive
Mobius syndromeOpen Targets
0.05Suggestive
Ptosis - vocal cord paralysisOpen Targets
0.05Suggestive
ptosis-vocal cord paralysis syndromeOpen Targets
0.05Suggestive
Cataract with Y-shaped suture opacitiesOpen Targets
0.04Suggestive
amyotrophic lateral sclerosis-parkinsonism-dementia complexOpen Targets
0.04Suggestive
MedulloblastomaUniProt
Pathogenic Variants2
NM_001375883.1(GPR161):c.585G>A (p.Trp195Ter)Likely pathogenic
Medulloblastoma
★☆☆☆2025→ Residue 195
NM_001375883.1(GPR161):c.487_488del (p.Leu163fs)Pathogenic
Medulloblastoma
☆☆☆☆2022→ Residue 163
View on ClinVar ↗
Related Genes
IFT122Protein interaction100%PRKACAProtein interaction97%FOXE3Protein interaction96%PRKACGProtein interaction95%PRKACBProtein interaction95%PRKAR1AProtein interaction94%
Tissue Expression6 tissues
Ovary
100%
Brain
68%
Heart
51%
Lung
42%
Liver
7%
Bone Marrow
4%
Gene Interaction Network
Click a node to explore
GPR161IFT122PRKACAFOXE3PRKACGPRKACBPRKAR1A
PROTEIN STRUCTURE
Preparing viewer…
PDB8SMV · 2.74 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.85LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.61 [0.45–0.85]
RankingsWhere GPR161 stands among ~20K protein-coding genes
  • #10,977of 20,598
    Most Researched35
  • #4,486of 5,498
    Most Pathogenic Variants2
  • #7,455of 17,882
    Most Constrained (LOEUF)0.85
Genes detectedGPR161
Sources retrieved10 papers
Response time—
📄 Sources
10â–¼
1
Rgl-exomiR-7972, a novel plant exosomal microRNA derived from fresh Rehmanniae Radix, ameliorated lipopolysaccharide-induced acute lung injury and gut dysbiosis.
PMID: 37327587
Biomed Pharmacother · 2023
1.00
2
GPR161 structure uncovers the redundant role of sterol-regulated ciliary cAMP signaling in the Hedgehog pathway.
PMID: 38326651
Nat Struct Mol Biol · 2024
0.90
3
GPR161 mechanosensitivity at the primary cilium drives neuronal saltatory migration.
PMID: 40737401
Sci Adv · 2025
0.80
4
The ancestral ESCRT protein TOM1L2 selects ubiquitinated cargoes for retrieval from cilia.
PMID: 37019113
Dev Cell · 2023
0.70
5
Sequence-structure based phylogeny of GPCR Class A Rhodopsin receptors.
PMID: 24503482
Mol Phylogenet Evol · 2014
0.60