P01148 (GON1_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Progonadoliberin-1

Alternative name(s):
Progonadoliberin I

Cleaved into the following 2 chains:

1. Gonadoliberin-1
   Alternative name(s):
   Gonadoliberin I
   Gonadorelin
   Gonadotropin-releasing hormone I
   Short name=GnRH-I
   Luliberin I
   Luteinizing hormone-releasing hormone I
   Short name=LH-RH I
2. GnRH-associated peptide 1
   Alternative name(s):
   GnRH-associated peptide I

Gene names

Name:	GNRH1
Synonyms:	GNRH, GRH, LHRH

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	92 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Stimulates the secretion of gonadotropins; it stimulates the secretion of both luteinizing and follicle-stimulating hormones.
Subcellular location	Secreted.
Pharmaceutical use	Available under the names Factrel (Ayerst Labs), Lutrepulse or Lutrelef (Ferring Pharmaceuticals) and Relisorm (Serono). Used in evaluating hypothalamic-pituitary gonadotropic function.
Sequence similarities	Belongs to the GnRH family.

Ontologies

Keywords
Cellular component	Secreted
Coding sequence diversity	Polymorphism
Domain	Signal
Molecular function	Hormone
PTM	Amidation Cleavage on pair of basic residues Pyrrolidone carboxylic acid
Technical term	Complete proteome Direct protein sequencing Pharmaceutical Reference proteome
Gene Ontology (GO)
Biological process	cell-cell signaling Traceable author statement. Source: ProtInc multicellular organismal development Traceable author statement. Source: ProtInc negative regulation of cell proliferation Traceable author statement. Source: ProtInc signal transduction Traceable author statement. Source: ProtInc
Cellular component	soluble fraction Traceable author statement. Source: ProtInc
Molecular function	gonadotropin hormone-releasing hormone activity Traceable author statement. Source: ProtInc
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Signal peptide	1 – 23	23	Ref.5
	Chain	24 – 92	69	Progonadoliberin-1		PRO_0000012395
	Peptide	24 – 33	10	Gonadoliberin-1 Ref.5		PRO_0000012396
	Peptide	37 – 92	56	GnRH-associated peptide 1		PRO_0000012397
Sites
	Site	26	1	Appears to be essential for biological activity
Amino acid modifications
	Modified residue	24	1	Pyrrolidone carboxylic acid Ref.5
	Modified residue	33	1	Glycine amide Ref.5
Natural variations
	Natural variant	16	1	W → S. Ref.3 Ref.6 Corresponds to variant rs6185 [ dbSNP | Ensembl ].		VAR_013943

Sequences

	Sequence		Length	Mass (Da)
	P01148 [UniParc]. Last modified April 1, 1988. Version 1. Checksum: 30A72221B076FA79	FASTA	92	10,380
	10 20 30 40 50 60 MKPIQKLLAG LILLTWCVEG CSSQHWSYGL RPGGKRDAEN LIDSFQEIVK EVGQLAETQR 70 80 90 FECTTHQPRS PLRDLKGALE SLIEEETGQK KI

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"The complete nucleotide sequence of the human gonadotropin-releasing hormone gene." Hayflick J.S., Adelman J.P., Seeburg P.H. Nucleic Acids Res. 17:6403-6403(1989) [PubMed: 2671939] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[2]	"Isolation of the gene and hypothalamic cDNA for the common precursor of gonadotropin-releasing hormone and prolactin release-inhibiting factor in human and rat." Adelman J.P., Mason A.J., Hayflick J.S., Seeburg P.H. Proc. Natl. Acad. Sci. U.S.A. 83:179-183(1986) [PubMed: 2867548] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[3]	"Characterization of cDNA for precursor of human luteinizing hormone releasing hormone." Seeburg P.H., Adelman J.P. Nature 311:666-668(1984) [PubMed: 6090951] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT SER-16.
[4]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Brain.
[5]	"The chemical identity of the immunoreactive LHRH-like peptide biosynthesized in the human placenta." Tan L., Rousseau P. Biochem. Biophys. Res. Commun. 109:1061-1071(1982) [PubMed: 6760865] [Abstract] Cited for: PROTEIN SEQUENCE OF 24-33.
[6]	"Characterization of single-nucleotide polymorphisms in coding regions of human genes." Cargill M., Altshuler D., Ireland J., Sklar P., Ardlie K., Patil N., Shaw N., Lane C.R., Lim E.P., Kalyanaraman N., Nemesh J., Ziaugra L., Friedland L., Rolfe A., Warrington J., Lipshutz R., Daley G.Q., Lander E.S. Nat. Genet. 22:231-238(1999) [PubMed: 10391209] [Abstract] Cited for: VARIANT SER-16.
[7]	Erratum Cargill M., Altshuler D., Ireland J., Sklar P., Ardlie K., Patil N., Shaw N., Lane C.R., Lim E.P., Kalyanaraman N., Nemesh J., Ziaugra L., Friedland L., Rolfe A., Warrington J., Lipshutz R., Daley G.Q., Lander E.S. Nat. Genet. 23:373-373(1999)
+	Additional computationally mapped references.

Web resources

Wikipedia

Gonadotropin-releasing hormone entry

Cross-references

Sequence databases
EMBL GenBank DDBJ	X01059 mRNA. Translation: CAA25526.1. M12578 mRNA. Translation: AAA35916.1. X15215 Genomic DNA. Translation: CAA33285.1. BC126437 mRNA. Translation: AAI26438.1. BC126463 mRNA. Translation: AAI26464.1.
IPI	IPI00000102.
PIR	RHHUG. S05308.
RefSeq	NP_000816.3. NM_000825.3. NP_001076580.1. NM_001083111.1.
UniGene	Hs.82963.
3D structure databases
ProteinModelPortal	P01148.
ModBase	Search...
Protein-protein interaction databases
STRING	P01148.
Polymorphism databases
DMDM	121522.
Proteomic databases
PRIDE	P01148.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000276414; ENSP00000276414; ENSG00000147437. ENST00000421054; ENSP00000391280; ENSG00000147437.
GeneID	2796.
KEGG	hsa:2796.
UCSC	uc003xem.2. human.
Organism-specific databases
CTD	2796.
GeneCards	GC08M025276.
H-InvDB	HIX0034347.
HGNC	HGNC:4419. GNRH1.
HPA	HPA027532.
MIM	152760. gene.
neXtProt	NX_P01148.
Orphanet	432. Normosmic congenital hypogonadotropic hypogonadism.
PharmGKB	PA28798.
GenAtlas	Search...
Phylogenomic databases
eggNOG	prNOG21351.
GeneTree	ENSGT00390000008225.
HOGENOM	HBG282417.
HOVERGEN	HBG014746.
InParanoid	P01148.
OMA	GCSSQHW.
OrthoDB	EOG4MCX1W.
PhylomeDB	P01148.
Enzyme and pathway databases
Reactome	REACT_111102. Signal Transduction.
Gene expression databases
ArrayExpress	P01148.
Bgee	P01148.
CleanEx	HS_GNRH1.
Genevestigator	P01148.
GermOnline	ENSG00000147437. Homo sapiens.
Family and domain databases
InterPro	IPR002012. GnRH. IPR019792. Gonadoliberin_I. IPR004079. Gonadoliberin_I_precursor. [Graphical view]
KO	K05252.
PANTHER	PTHR10522. GnRH. 1 hit.
Pfam	PF00446. GnRH. 1 hit. [Graphical view]
PRINTS	PR01541. GONADOLIBRNI.
PROSITE	PS00473. GNRH. 1 hit. [Graphical view]
ProtoNet	Search...
Other
NextBio	11013.
PMAP-CutDB	P01148.
SOURCE	Search...

Entry information

Entry name

GON1_HUMAN

Accession

Primary (citable) accession number: P01148
Secondary accession number(s): A0AVP0

Entry history

Integrated into UniProtKB/Swiss-Prot:	July 21, 1986
Last sequence update:	April 1, 1988
Last modified:	January 25, 2012
This is version 120 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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.