GNRH1

Functionⁱ

Stimulates the secretion of gonadotropins; it stimulates the secretion of both luteinizing and follicle-stimulating hormones.

Miscellaneous

Sites

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Siteⁱ	26	Appears to be essential for biological activity		1

GO - Molecular functionⁱ

gonadotropin hormone-releasing hormone activity
Source: ProtInc
Traceable author statementⁱ
- 10832105
hormone activity
Source: ProtInc
Traceable author statementⁱ
- 2863757

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

aging Source: Ensembl
cell-cell signaling
Source: ProtInc
Traceable author statementⁱ
- 1310542
estrous cycle Source: Ensembl
female pregnancy Source: Ensembl
G-protein coupled receptor signaling pathway Source: Reactome
male sex determination Source: Ensembl
multicellular organism development
Source: ProtInc
Traceable author statementⁱ
- 2867548
negative regulation of apoptotic process Source: Ensembl
negative regulation of cell proliferation
Source: ProtInc
Traceable author statementⁱ
- 10832105
negative regulation of immature T cell proliferation Source: Ensembl
negative regulation of neuron migration Source: Ensembl
regulation of gene expression Source: Ensembl
regulation of ovarian follicle development Source: Ensembl
response to corticosteroid Source: Ensembl
response to ethanol Source: Ensembl
response to lipopolysaccharide Source: Ensembl
response to potassium ion Source: Ensembl
response to prolactin Source: Ensembl
response to prostaglandin E Source: Ensembl
response to testosterone Source: Ensembl
signal transduction
Source: ProtInc
Traceable author statementⁱ
- 8692981

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Hormone

Molecular function

Hormone

Enzyme and pathway databases

Reactomeⁱ	R-HSA-375281 Hormone ligand-binding receptors R-HSA-416476 G alpha (q) signalling events
SIGNORⁱ	P01148

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Progonadoliberin-1 Alternative name(s): Progonadoliberin I Cleaved into the following 2 chains: 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 GnRH-associated peptide 1 Alternative name(s): GnRH-associated peptide I
Gene namesⁱ	Name:GNRH1 Synonyms:GNRH, GRH, LHRH
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 8

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000147437.9
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:4419 GNRH1
MIMⁱ	152760 gene
neXtProtⁱ	NX_P01148

Keywords - Cellular componentⁱ

Secreted

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Hypogonadotropic hypogonadism 12 with or without anosmia (HH12)2 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 GNRH1 as well as in other HH-associated genes including PROKR2 and FGFR1 (PubMed:23643382).1 Publication

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).

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_069966	31	R → C in HH12; uncertain pathological significance; the patient also carries mutations in PROKR2 and FGFR1. 1 Publication		1

Pharmaceutical useⁱ

Available under the names Factrel (Ayerst Labs), Lutrepulse or Lutrelef (Ferring Pharmaceuticals) and Relisorm (Serono). Used in evaluating hypothalamic-pituitary gonadotropic function.

Keywords - Diseaseⁱ

Disease mutation, Hypogonadotropic hypogonadism, Kallmann syndrome

Organism-specific databases

DisGeNET More...DisGeNETⁱ	2796
GeneReviewsⁱ	GNRH1
MalaCards human disease database More...MalaCardsⁱ	GNRH1
MIMⁱ	614841 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000147437
Orphanetⁱ	432 Normosmic congenital hypogonadotropic hypogonadism
PharmGKBⁱ	PA28798

Polymorphism and mutation databases

BioMutaⁱ	GNRH1
DMDMⁱ	121522

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Length
Signal peptideⁱ	1 – 23	1 PublicationAdd BLAST	23
ChainⁱPRO_0000012395	24 – 92	Progonadoliberin-1Add BLAST	69
PeptideⁱPRO_0000012396	24 – 33	Gonadoliberin-11 Publication	10
PeptideⁱPRO_0000012397	37 – 92	GnRH-associated peptide 1Add BLAST	56

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Modified residueⁱ	24	Pyrrolidone carboxylic acid1 Publication		1
Modified residueⁱ	33	Glycine amide1 Publication		1

Keywords - PTMⁱ

Amidation, Cleavage on pair of basic residues, Pyrrolidone carboxylic acid

Proteomic databases

PaxDbⁱ	P01148
PeptideAtlas More...PeptideAtlasⁱ	P01148
PRIDEⁱ	P01148
ProteomicsDBⁱ	51339

Miscellaneous databases

PMAP-CutDBⁱ	P01148

PMAP-CutDBⁱ

P01148

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000147437
CleanExⁱ	HS_GNRH1
Genevisibleⁱ	P01148 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA027532

HPAⁱ

HPA027532

Interactionⁱ

GO - Molecular functionⁱ

gonadotropin hormone-releasing hormone activity
Source: ProtInc
Traceable author statementⁱ
- 10832105
hormone activity
Source: ProtInc
Traceable author statementⁱ
- 2863757

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridⁱ	109058, 8 interactors
STRINGⁱ	9606.ENSP00000276414

Structureⁱ

Secondary structure

Legend: HelixTurnBeta strandPDB Structure known for this area

Show more details

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Helixⁱ	26 – 28	Combined sources		3

3D structure databases

ProteinModelPortalⁱ	P01148
SMRⁱ	P01148
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Sequence similaritiesⁱ

Belongs to the GnRH family.Curated

Keywords - Domainⁱ

Signal

Phylogenomic databases

eggNOGⁱ	ENOG410J18B Eukaryota ENOG410ZDKS LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000008225
HOGENOMⁱ	HOG000033698
HOVERGENⁱ	HBG014746
InParanoidⁱ	P01148
KEGG Orthology (KO) More...KOⁱ	K05252
OMAⁱ	EPQRFEC
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G147V
PhylomeDBⁱ	P01148
TreeFamⁱ	TF330934

Family and domain databases

InterProⁱ	View protein in InterPro IPR002012 GnRH IPR019792 Gonadoliberin_I IPR004079 Gonadoliberin_I_precursor
PANTHERⁱ	PTHR10522 PTHR10522, 1 hit PTHR10522:SF0 PTHR10522:SF0, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF00446 GnRH, 1 hit
PRINTSⁱ	PR01541 GONADOLIBRNI
PROSITEⁱ	View protein in PROSITE PS00473 GNRH, 1 hit

Sequenceⁱ

Sequence statusⁱ: Complete.

Sequence processingⁱ: The displayed sequence is further processed into a mature form.

P01148-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MKPIQKLLAG LILLTWCVEG CSSQHWSYGL RPGGKRDAEN LIDSFQEIVK 
        60         70         80         90 
EVGQLAETQR FECTTHQPRS PLRDLKGALE SLIEEETGQK KI

Length:92

Mass (Da):10,380

Last modified:April 1, 1988 - v1

Checksum:ⁱ30A72221B076FA79

GO

Natural variant

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_013943	16	W → S2 PublicationsCorresponds to variant dbSNP:rs6185Ensembl.		1
Natural variantⁱVAR_069966	31	R → C in HH12; uncertain pathological significance; the patient also carries mutations in PROKR2 and FGFR1. 1 Publication		1

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	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
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS43725.1
PIRⁱ	S05308 RHHUG
NCBI Reference Sequences More...RefSeqⁱ	NP_001076580.1, NM_001083111.1
UniGeneⁱ	Hs.82963

Genome annotation databases

Ensemblⁱ	ENST00000276414; ENSP00000276414; ENSG00000147437 ENST00000421054; ENSP00000391280; ENSG00000147437
GeneIDⁱ	2796
KEGGⁱ	hsa:2796
UCSC genome browser More...UCSCⁱ	uc003xem.5 human

Keywords - Coding sequence diversityⁱ

Polymorphism

Similar proteinsⁱ

90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length
P01148	UPI0000D4C062		HUMAN		96
Gonadoliberin		PANTR		96
Gonadoliberin		PANPA		96
Gonadoliberin		GORGO		96
Gonadoliberin		PANTR		92

Protein	Similar proteins		Species	Score	Length
P01148	UPI0000D4C062		HUMAN		96
Gonadoliberin		GORGO		96
Gonadoliberin		PANTR		96
Gonadoliberin		PANPA		96
Gonadoliberin		ERIEU		100
+102

Cross-referencesⁱ

Web resourcesⁱ

Wikipedia

Gonadotropin-releasing hormone entry

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	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
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS43725.1
PIRⁱ	S05308 RHHUG
NCBI Reference Sequences More...RefSeqⁱ	NP_001076580.1, NM_001083111.1
UniGeneⁱ	Hs.82963

3D structure databases

Select the link destinations: Protein Data Bank Europe More...PDBeⁱ Protein Data Bank RCSB More...RCSB PDBⁱ Protein Data Bank Japan More...PDBjⁱ Links Updated	PDB entry	Method	Resolution (Å)	Chain	Positions	PDBsum
	4D5M	X-ray	0.85	A/B/C/D	24-33	[»]
ProteinModelPortalⁱ	P01148
SMRⁱ	P01148
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	109058, 8 interactors
STRINGⁱ	9606.ENSP00000276414

Polymorphism and mutation databases

BioMutaⁱ	GNRH1
DMDMⁱ	121522

Proteomic databases

PaxDbⁱ	P01148
PeptideAtlas More...PeptideAtlasⁱ	P01148
PRIDEⁱ	P01148
ProteomicsDBⁱ	51339

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	2796
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000276414; ENSP00000276414; ENSG00000147437 ENST00000421054; ENSP00000391280; ENSG00000147437
GeneIDⁱ	2796
KEGGⁱ	hsa:2796
UCSC genome browser More...UCSCⁱ	uc003xem.5 human

Organism-specific databases

CTDⁱ	2796
DisGeNET More...DisGeNETⁱ	2796
EuPathDBⁱ	HostDB:ENSG00000147437.9
GeneCardsⁱ	GNRH1
GeneReviewsⁱ	GNRH1
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:4419 GNRH1
Human Protein Atlas More...HPAⁱ	HPA027532
MalaCards human disease database More...MalaCardsⁱ	GNRH1
MIMⁱ	152760 gene 614841 phenotype
neXtProtⁱ	NX_P01148
Open Targets More...OpenTargetsⁱ	ENSG00000147437
Orphanetⁱ	432 Normosmic congenital hypogonadotropic hypogonadism
PharmGKBⁱ	PA28798
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	ENOG410J18B Eukaryota ENOG410ZDKS LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000008225
HOGENOMⁱ	HOG000033698
HOVERGENⁱ	HBG014746
InParanoidⁱ	P01148
KEGG Orthology (KO) More...KOⁱ	K05252
OMAⁱ	EPQRFEC
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G147V
PhylomeDBⁱ	P01148
TreeFamⁱ	TF330934

Enzyme and pathway databases

Reactomeⁱ	R-HSA-375281 Hormone ligand-binding receptors R-HSA-416476 G alpha (q) signalling events
SIGNORⁱ	P01148

Miscellaneous databases

GeneWikiⁱ	Gonadotropin-releasing_hormone
GenomeRNAiⁱ	2796
PMAP-CutDBⁱ	P01148
Protein Ontology More...PROⁱ	PR:P01148
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000147437
CleanExⁱ	HS_GNRH1
Genevisibleⁱ	P01148 HS

Family and domain databases

InterProⁱ	View protein in InterPro IPR002012 GnRH IPR019792 Gonadoliberin_I IPR004079 Gonadoliberin_I_precursor
PANTHERⁱ	PTHR10522 PTHR10522, 1 hit PTHR10522:SF0 PTHR10522:SF0, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF00446 GnRH, 1 hit
PRINTSⁱ	PR01541 GONADOLIBRNI
PROSITEⁱ	View protein in PROSITE PS00473 GNRH, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	GON1_HUMAN
Accessionⁱ	P01148Primary (citable) accession number: P01148 Secondary accession number(s): A0AVP0
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	July 21, 1986
	Last sequence update:	April 1, 1988
	Last modified:	June 20, 2018
	This is version 170 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	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.

Miscellaneousⁱ

Keywords - Technical termⁱ

3D-structure, Complete proteome, Direct protein sequencing, Pharmaceutical, Reference proteome

Documents

Human chromosome 8
Human chromosome 8: entries, gene names and cross-references to MIM
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
PDB cross-references
Index of Protein Data Bank (PDB) cross-references
SIMILARITY comments
Index of protein domains and families

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UniProtKB - P01148 (GON1_HUMAN)

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Progonadoliberin-1

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Miscellaneous

Sites

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted

Endoplasmic reticulum

Extracellular region or secreted

Mitochondrion

Other locations

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Organism-specific databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Proteomic databases

Miscellaneous databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Phylogenomic databases

Family and domain databases

Natural variant

Sequence databases

Genome annotation databases

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Sequence databases

3D structure databases

Protein-protein interaction databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Cross-referencesⁱ

Web resourcesⁱ

Entry informationⁱ

Miscellaneousⁱ