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Protein

Prokineticin receptor 2

Gene

PROKR2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Receptor for prokineticin 2. Exclusively coupled to the G(q) subclass of heteromeric G proteins. Activation leads to mobilization of calcium, stimulation of phosphoinositide turnover and activation of p44/p42 mitogen-activated protein kinase.

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

G-protein coupled receptor, Receptor, Transducer

Enzyme and pathway databases

BioCyciZFISH:ENSG00000101292-MONOMER.
ReactomeiR-HSA-375276. Peptide ligand-binding receptors.
R-HSA-416476. G alpha (q) signalling events.
SIGNORiQ8NFJ6.

Names & Taxonomyi

Protein namesi
Recommended name:
Prokineticin receptor 2
Short name:
PK-R2
Alternative name(s):
G-protein coupled receptor 73-like 1
G-protein coupled receptor I5E
GPR73b
GPRg2
Gene namesi
Name:PROKR2
Synonyms:GPR73L1, PKR2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 20

Organism-specific databases

HGNCiHGNC:15836. PROKR2.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 54ExtracellularSequence analysisAdd BLAST54
Transmembranei55 – 75Helical; Name=1Sequence analysisAdd BLAST21
Topological domaini76 – 89CytoplasmicSequence analysisAdd BLAST14
Transmembranei90 – 110Helical; Name=2Sequence analysisAdd BLAST21
Topological domaini111 – 136ExtracellularSequence analysisAdd BLAST26
Transmembranei137 – 157Helical; Name=3Sequence analysisAdd BLAST21
Topological domaini158 – 171CytoplasmicSequence analysisAdd BLAST14
Transmembranei172 – 192Helical; Name=4Sequence analysisAdd BLAST21
Topological domaini193 – 223ExtracellularSequence analysisAdd BLAST31
Transmembranei224 – 244Helical; Name=5Sequence analysisAdd BLAST21
Topological domaini245 – 273CytoplasmicSequence analysisAdd BLAST29
Transmembranei274 – 294Helical; Name=6Sequence analysisAdd BLAST21
Topological domaini295 – 313ExtracellularSequence analysisAdd BLAST19
Transmembranei314 – 334Helical; Name=7Sequence analysisAdd BLAST21
Topological domaini335 – 384CytoplasmicSequence analysisAdd BLAST50

GO - Cellular componenti

  • integral component of plasma membrane Source: GO_Central
  • plasma membrane Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cell membrane, Membrane

Pathology & Biotechi

Involvement in diseasei

Hypogonadotropic hypogonadism 3 with or without anosmia (HH3)6 Publications
The disease is caused by mutations affecting distinct genetic loci, including the gene represented in this entry. The genetics of hypogonadotropic hypogonadism involves various modes of transmission. Oligogenic inheritance has been reported in some patients carrying mutations in PROKR2 as well as in other HH-associated genes including KAL1, SEMA3A, PROK2, GNRH1 and FGFR1 (PubMed:17054399, PubMed:22927827, PubMed:23643382).3 Publications
Disease descriptionA disorder characterized by absent or incomplete sexual maturation by the age of 18 years, in conjunction with low levels of circulating gonadotropins and testosterone and no other abnormalities of the hypothalamic-pituitary axis. In some cases, it is associated with non-reproductive phenotypes, such as anosmia, cleft palate, and sensorineural hearing loss. Anosmia or hyposmia is related to the absence or hypoplasia of the olfactory bulbs and tracts. Hypogonadism is due to deficiency in gonadotropin-releasing hormone and probably results from a failure of embryonic migration of gonadotropin-releasing hormone-synthesizing neurons. In the presence of anosmia, idiopathic hypogonadotropic hypogonadism is referred to as Kallmann syndrome, whereas in the presence of a normal sense of smell, it has been termed normosmic idiopathic hypogonadotropic hypogonadism (nIHH).
See also OMIM:244200
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03095785R → C in HH3; phenotype consistent with normosmic idiopathic hypogonadotropic hypogonadism; decreased signaling activity. 4 PublicationsCorresponds to variant rs141090506dbSNPEnsembl.1
Natural variantiVAR_03095885R → H in HH3; decreased signaling activity. 3 PublicationsCorresponds to variant rs74315418dbSNPEnsembl.1
Natural variantiVAR_072173113Y → H in HH3. 1 PublicationCorresponds to variant rs202203360dbSNPEnsembl.1
Natural variantiVAR_069964115V → M in HH3; phenotype consistent with Kallmann syndrome. 2 PublicationsCorresponds to variant rs138672528dbSNPEnsembl.1
Natural variantiVAR_072978158V → I in HH3; phenotype consistent with Kallmann syndrome. 1 PublicationCorresponds to variant rs368732206dbSNPEnsembl.1
Natural variantiVAR_030959164R → Q in HH3; phenotype consistent with Kallmann syndrome; decreased signaling activity. 3 Publications1
Natural variantiVAR_030960173L → R in HH3; phenotype consistent with Kallmann syndrome; decreased signaling activity. 4 PublicationsCorresponds to variant rs74315416dbSNPEnsembl.1
Natural variantiVAR_030961178W → S in HH3; decreased signaling activity. 3 PublicationsCorresponds to variant rs201835496dbSNPEnsembl.1
Natural variantiVAR_072174188S → L in HH3. 1 PublicationCorresponds to variant rs376239580dbSNPEnsembl.1
Natural variantiVAR_069965202S → G in HH3; triallelic inheritance; the patient also carries mutations in GNRH1 and FGFR1. 1 PublicationCorresponds to variant rs200755554dbSNPEnsembl.1
Natural variantiVAR_030962210Q → R in HH3; phenotype consistent with Kallmann syndrome; decreased signaling activity; abolished ligand binding. 2 PublicationsCorresponds to variant rs74315417dbSNPEnsembl.1
Natural variantiVAR_072175248R → Q in HH3. 1 PublicationCorresponds to variant rs376142095dbSNPEnsembl.1
Natural variantiVAR_030963268R → C in HH3; uncertain pathological significance; signaling activity is impaired. 3 PublicationsCorresponds to variant rs78861628dbSNPEnsembl.1
Natural variantiVAR_030964290P → S in HH3; phenotype consistent with Kallmann syndrome; signaling activity is impaired; impaired cell surface-targeting. 3 PublicationsCorresponds to variant rs149992595dbSNPEnsembl.1
Natural variantiVAR_030965323M → I in HH3; phenotype consistent with Kallmann syndrome; signaling activity is impaired. 2 PublicationsCorresponds to variant rs74315419dbSNPEnsembl.1
Natural variantiVAR_030966331V → M in HH3; uncertain pathological significance. 4 PublicationsCorresponds to variant rs117106081dbSNPEnsembl.1
Natural variantiVAR_072979334V → M in HH3; phenotype consistent with Kallmann syndrome. 1 PublicationCorresponds to variant rs371564610dbSNPEnsembl.1
Natural variantiVAR_072176357R → W in HH3. 1 PublicationCorresponds to variant rs375036628dbSNPEnsembl.1

Keywords - Diseasei

Disease mutation, Hypogonadotropic hypogonadism, Kallmann syndrome

Organism-specific databases

DisGeNETi128674.
MalaCardsiPROKR2.
MIMi244200. phenotype.
OpenTargetsiENSG00000101292.
Orphaneti478. Kallmann syndrome.
432. Normosmic congenital hypogonadotropic hypogonadism.
3157. Septo-optic dysplasia.
PharmGKBiPA30014.

Chemistry databases

ChEMBLiCHEMBL5548.
GuidetoPHARMACOLOGYi336.

Polymorphism and mutation databases

BioMutaiPROKR2.
DMDMi33112425.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000700831 – 384Prokineticin receptor 2Add BLAST384

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi7N-linked (GlcNAc...)Sequence analysis1
Glycosylationi27N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi128 ↔ 208PROSITE-ProRule annotation

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiQ8NFJ6.
PRIDEiQ8NFJ6.

PTM databases

iPTMnetiQ8NFJ6.
PhosphoSitePlusiQ8NFJ6.

Expressioni

Tissue specificityi

Expressed in the ileocecum, thyroid gland, pituitary gland, salivary gland, adrenal gland, testis, ovary and brain.

Gene expression databases

BgeeiENSG00000101292.
CleanExiHS_PROKR2.
GenevisibleiQ8NFJ6. HS.

Organism-specific databases

HPAiHPA047281.

Interactioni

Subunit structurei

Homodimer.1 Publication

Protein-protein interaction databases

BioGridi126143. 1 interactor.
STRINGi9606.ENSP00000217270.

Chemistry databases

BindingDBiQ8NFJ6.

Structurei

3D structure databases

ProteinModelPortaliQ8NFJ6.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the G-protein coupled receptor 1 family.PROSITE-ProRule annotation

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3656. Eukaryota.
ENOG410XRW9. LUCA.
GeneTreeiENSGT00820000127076.
HOGENOMiHOG000231664.
HOVERGENiHBG039631.
InParanoidiQ8NFJ6.
KOiK08380.
OMAiDYDLPMD.
OrthoDBiEOG091G086Y.
PhylomeDBiQ8NFJ6.
TreeFamiTF315303.

Family and domain databases

InterProiIPR000276. GPCR_Rhodpsn.
IPR017452. GPCR_Rhodpsn_7TM.
IPR000611. NPY_rcpt.
[Graphical view]
PfamiPF00001. 7tm_1. 1 hit.
[Graphical view]
PRINTSiPR00237. GPCRRHODOPSN.
PR01012. NRPEPTIDEYR.
PROSITEiPS00237. G_PROTEIN_RECEP_F1_1. 1 hit.
PS50262. G_PROTEIN_RECEP_F1_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Q8NFJ6-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAAQNGNTSF TPNFNPPQDH ASSLSFNFSY GDYDLPMDED EDMTKTRTFF
60 70 80 90 100
AAKIVIGIAL AGIMLVCGIG NFVFIAALTR YKKLRNLTNL LIANLAISDF
110 120 130 140 150
LVAIICCPFE MDYYVVRQLS WEHGHVLCAS VNYLRTVSLY VSTNALLAIA
160 170 180 190 200
IDRYLAIVHP LKPRMNYQTA SFLIALVWMV SILIAIPSAY FATETVLFIV
210 220 230 240 250
KSQEKIFCGQ IWPVDQQLYY KSYFLFIFGV EFVGPVVTMT LCYARISREL
260 270 280 290 300
WFKAVPGFQT EQIRKRLRCR RKTVLVLMCI LTAYVLCWAP FYGFTIVRDF
310 320 330 340 350
FPTVFVKEKH YLTAFYVVEC IAMSNSMINT VCFVTVKNNT MKYFKKMMLL
360 370 380
HWRPSQRGSK SSADLDLRTN GVPTTEEVDC IRLK
Length:384
Mass (Da):43,996
Last modified:October 1, 2002 - v1
Checksum:i2D5BFA3655347B5E
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03095785R → C in HH3; phenotype consistent with normosmic idiopathic hypogonadotropic hypogonadism; decreased signaling activity. 4 PublicationsCorresponds to variant rs141090506dbSNPEnsembl.1
Natural variantiVAR_03095885R → H in HH3; decreased signaling activity. 3 PublicationsCorresponds to variant rs74315418dbSNPEnsembl.1
Natural variantiVAR_072173113Y → H in HH3. 1 PublicationCorresponds to variant rs202203360dbSNPEnsembl.1
Natural variantiVAR_069964115V → M in HH3; phenotype consistent with Kallmann syndrome. 2 PublicationsCorresponds to variant rs138672528dbSNPEnsembl.1
Natural variantiVAR_072978158V → I in HH3; phenotype consistent with Kallmann syndrome. 1 PublicationCorresponds to variant rs368732206dbSNPEnsembl.1
Natural variantiVAR_030959164R → Q in HH3; phenotype consistent with Kallmann syndrome; decreased signaling activity. 3 Publications1
Natural variantiVAR_030960173L → R in HH3; phenotype consistent with Kallmann syndrome; decreased signaling activity. 4 PublicationsCorresponds to variant rs74315416dbSNPEnsembl.1
Natural variantiVAR_030961178W → S in HH3; decreased signaling activity. 3 PublicationsCorresponds to variant rs201835496dbSNPEnsembl.1
Natural variantiVAR_072174188S → L in HH3. 1 PublicationCorresponds to variant rs376239580dbSNPEnsembl.1
Natural variantiVAR_069965202S → G in HH3; triallelic inheritance; the patient also carries mutations in GNRH1 and FGFR1. 1 PublicationCorresponds to variant rs200755554dbSNPEnsembl.1
Natural variantiVAR_030962210Q → R in HH3; phenotype consistent with Kallmann syndrome; decreased signaling activity; abolished ligand binding. 2 PublicationsCorresponds to variant rs74315417dbSNPEnsembl.1
Natural variantiVAR_072175248R → Q in HH3. 1 PublicationCorresponds to variant rs376142095dbSNPEnsembl.1
Natural variantiVAR_030963268R → C in HH3; uncertain pathological significance; signaling activity is impaired. 3 PublicationsCorresponds to variant rs78861628dbSNPEnsembl.1
Natural variantiVAR_030964290P → S in HH3; phenotype consistent with Kallmann syndrome; signaling activity is impaired; impaired cell surface-targeting. 3 PublicationsCorresponds to variant rs149992595dbSNPEnsembl.1
Natural variantiVAR_030965323M → I in HH3; phenotype consistent with Kallmann syndrome; signaling activity is impaired. 2 PublicationsCorresponds to variant rs74315419dbSNPEnsembl.1
Natural variantiVAR_030966331V → M in HH3; uncertain pathological significance. 4 PublicationsCorresponds to variant rs117106081dbSNPEnsembl.1
Natural variantiVAR_072979334V → M in HH3; phenotype consistent with Kallmann syndrome. 1 PublicationCorresponds to variant rs371564610dbSNPEnsembl.1
Natural variantiVAR_030967335T → M.1 PublicationCorresponds to variant rs755562438dbSNPEnsembl.1
Natural variantiVAR_072176357R → W in HH3. 1 PublicationCorresponds to variant rs375036628dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF506288 mRNA. Translation: AAM48128.1.
AB084081 mRNA. Translation: BAC24022.1.
EF577398 mRNA. Translation: ABQ52418.1.
AL121755 Genomic DNA. Translation: CAI22379.1.
BC104959 mRNA. Translation: AAI04960.1.
BC104961 mRNA. Translation: AAI04962.1.
CCDSiCCDS13089.1.
RefSeqiNP_658986.1. NM_144773.3.
XP_016883135.1. XM_017027646.1.
UniGeneiHs.375029.

Genome annotation databases

EnsembliENST00000217270; ENSP00000217270; ENSG00000101292.
GeneIDi128674.
KEGGihsa:128674.
UCSCiuc010zqw.3. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF506288 mRNA. Translation: AAM48128.1.
AB084081 mRNA. Translation: BAC24022.1.
EF577398 mRNA. Translation: ABQ52418.1.
AL121755 Genomic DNA. Translation: CAI22379.1.
BC104959 mRNA. Translation: AAI04960.1.
BC104961 mRNA. Translation: AAI04962.1.
CCDSiCCDS13089.1.
RefSeqiNP_658986.1. NM_144773.3.
XP_016883135.1. XM_017027646.1.
UniGeneiHs.375029.

3D structure databases

ProteinModelPortaliQ8NFJ6.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi126143. 1 interactor.
STRINGi9606.ENSP00000217270.

Chemistry databases

BindingDBiQ8NFJ6.
ChEMBLiCHEMBL5548.
GuidetoPHARMACOLOGYi336.

Protein family/group databases

GPCRDBiSearch...

PTM databases

iPTMnetiQ8NFJ6.
PhosphoSitePlusiQ8NFJ6.

Polymorphism and mutation databases

BioMutaiPROKR2.
DMDMi33112425.

Proteomic databases

PaxDbiQ8NFJ6.
PRIDEiQ8NFJ6.

Protocols and materials databases

DNASUi128674.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000217270; ENSP00000217270; ENSG00000101292.
GeneIDi128674.
KEGGihsa:128674.
UCSCiuc010zqw.3. human.

Organism-specific databases

CTDi128674.
DisGeNETi128674.
GeneCardsiPROKR2.
GeneReviewsiPROKR2.
HGNCiHGNC:15836. PROKR2.
HPAiHPA047281.
MalaCardsiPROKR2.
MIMi244200. phenotype.
607123. gene.
neXtProtiNX_Q8NFJ6.
OpenTargetsiENSG00000101292.
Orphaneti478. Kallmann syndrome.
432. Normosmic congenital hypogonadotropic hypogonadism.
3157. Septo-optic dysplasia.
PharmGKBiPA30014.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3656. Eukaryota.
ENOG410XRW9. LUCA.
GeneTreeiENSGT00820000127076.
HOGENOMiHOG000231664.
HOVERGENiHBG039631.
InParanoidiQ8NFJ6.
KOiK08380.
OMAiDYDLPMD.
OrthoDBiEOG091G086Y.
PhylomeDBiQ8NFJ6.
TreeFamiTF315303.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000101292-MONOMER.
ReactomeiR-HSA-375276. Peptide ligand-binding receptors.
R-HSA-416476. G alpha (q) signalling events.
SIGNORiQ8NFJ6.

Miscellaneous databases

GeneWikiiProkineticin_receptor_2.
GenomeRNAii128674.
PROiQ8NFJ6.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000101292.
CleanExiHS_PROKR2.
GenevisibleiQ8NFJ6. HS.

Family and domain databases

InterProiIPR000276. GPCR_Rhodpsn.
IPR017452. GPCR_Rhodpsn_7TM.
IPR000611. NPY_rcpt.
[Graphical view]
PfamiPF00001. 7tm_1. 1 hit.
[Graphical view]
PRINTSiPR00237. GPCRRHODOPSN.
PR01012. NRPEPTIDEYR.
PROSITEiPS00237. G_PROTEIN_RECEP_F1_1. 1 hit.
PS50262. G_PROTEIN_RECEP_F1_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiPKR2_HUMAN
AccessioniPrimary (citable) accession number: Q8NFJ6
Secondary accession number(s): A5JUU1
, Q2M3C0, Q5TDY1, Q9NTT0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 19, 2003
Last sequence update: October 1, 2002
Last modified: November 2, 2016
This is version 131 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. 7-transmembrane G-linked receptors
    List of 7-transmembrane G-linked receptor entries
  2. Human chromosome 20
    Human chromosome 20: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.