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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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NPFFR1
neuropeptide FF receptor 1
Chromosome 10 · 10q22.1
NCBI Gene: 64106Ensembl: ENSG00000148734.8HGNC: HGNC:17425UniProt: Q9GZQ6
17PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Receptor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingplasma membraneneuropeptide receptor activityciliumneurodegenerative disease46,XX gonadal dysgenesisprimary ovarian insufficiencypremature ovarian failure 17
✦AI Summary

NPFFR1 (neuropeptide FF receptor 1) is a G-protein-coupled receptor that primarily binds neuropeptide FF (NPFF) and RFamide-related peptide 3 (RFRP-3), mediating diverse physiological functions through Gαi/o signaling pathways that inhibit cAMP production 1. The receptor exhibits distinct ligand selectivity patterns, with structural studies revealing that the C-terminal RF-amide motif binds conserved transmembrane residues while N-terminal peptide regions determine receptor subtype specificity 2. NPFFR1 plays critical roles in cardiovascular regulation, with renal expression contributing to blood pressure control through antagonistic interactions with dopamine D1-like receptors in renal proximal tubule cells 3. The receptor is also involved in neuroendocrine regulation, particularly prolactin secretion, where RFRP-3 binding to NPFFR1 stimulates prolactin release independently of tuberoinfundibular dopaminergic neuron inhibition 4. NPFFR1 has therapeutic potential for treating pain, opioid tolerance, metabolic disorders, and hypertension 5. The receptor represents an evolutionarily conserved system within the RFamide peptide family, with structural similarities to invertebrate SIFamide receptors suggesting ancient origins 6. Understanding NPFFR1's selective ligand recognition mechanisms provides opportunities for developing targeted therapeutics with enhanced specificity.

Sources cited
1
NPFFR1 couples to Gαi/o proteins and inhibits cAMP production, with roles in cardiovascular regulation
PMID: 39769048
2
Structural basis of peptide recognition showing C-terminal RF-amide binding to conserved sites and N-terminal regions determining selectivity
PMID: 40839429
3
NPFFR1 expression in renal proximal tubules regulates blood pressure through antagonistic interactions with dopamine receptors
PMID: 38965251
4
RFRP-3 binding to NPFFR1 stimulates prolactin secretion independent of dopaminergic neuron inhibition
PMID: 37403228
5
NPFFR1 has therapeutic applications for pain, opioid side effects, and other conditions
PMID: 32673481
6
Evolutionary conservation of NPFFR1 within the RFamide peptide receptor family
PMID: 25220854
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.26Weak
46,XX gonadal dysgenesisOpen Targets
0.07Suggestive
primary ovarian insufficiencyOpen Targets
0.07Suggestive
premature ovarian failure 17Open Targets
0.05Suggestive
Combined pituitary hormone deficiencies, genetic formsOpen Targets
0.05Suggestive
Hypergonadotropic hypogonadism - cataract syndromeOpen Targets
0.05Suggestive
hypergonadotropic hypogonadism-cataract syndromeOpen Targets
0.05Suggestive
Kallmann syndromeOpen Targets
0.05Suggestive
spermatogenic failure 14Open Targets
0.05Suggestive
spermatogenic failure 13Open Targets
0.05Suggestive
premature ovarian failure 24Open Targets
0.04Suggestive
genetic non-acquired premature ovarian failureOpen Targets
0.04Suggestive
spermatogenic failure 77Open Targets
0.04Suggestive
spermatogenic failure, X-linked, 4Open Targets
0.04Suggestive
premature ovarian failure 23Open Targets
0.04Suggestive
ovarian dysgenesis 11Open Targets
0.04Suggestive
azoospermiaOpen Targets
0.04Suggestive
spermatogenic failure 28Open Targets
0.04Suggestive
premature ovarian failure 20Open Targets
0.04Suggestive
ovarian dysgenesis 10Open Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
QRFPRShared pathway100%KISS1Protein interaction98%NPFFR2Protein interaction79%OPRD1Protein interaction77%QRFPProtein interaction76%NPFFProtein interaction76%
Tissue Expression6 tissues
Brain
100%
Ovary
25%
Lung
22%
Liver
21%
Heart
5%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
NPFFR1QRFPRKISS1NPFFR2OPRD1QRFPNPFF
PROTEIN STRUCTURE
Preparing viewer…
PDB9M0R · 2.47 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.89LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.41 [0.95–1.89]
RankingsWhere NPFFR1 stands among ~20K protein-coding genes
  • #15,074of 20,598
    Most Researched17
  • #17,233of 17,882
    Most Constrained (LOEUF)1.89
Genes detectedNPFFR1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
An Overview on Renal and Central Regulation of Blood Pressure by Neuropeptide FF and Its Receptors.
PMID: 39769048
Int J Mol Sci · 2024
1.00
2
RFamide-related Peptide 3 Signaling via Neuropeptide FF Receptor Stimulates Prolactin Secretion in Female Rats.
PMID: 37403228
Endocrinology · 2023
0.90
3
Structural basis of peptide recognition and modulation for neuropeptide FF receptors.
PMID: 40839429
Cell Rep · 2025
0.80
4
Evolution of gonadotropin-inhibitory hormone receptor and its ligand.
PMID: 25220854
Gen Comp Endocrinol · 2014
0.70
5
Renal autocrine neuropeptide FF (NPFF) signaling regulates blood pressure.
PMID: 38965251
Sci Rep · 2024
0.60