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Details on Person UniProt:P01148 GNRH1

Class:IdReferenceGeneProduct:55788
_chainChangeLogsignal peptide:1-23 added on Fri February 6 2015;chain:24-92 added on Fri February 6 2015;peptide:24-33 added on Fri February 6 2015;peptide:37-92 added on Fri February 6 2015
_displayNameUniProt:P01148 GNRH1
_timestamp2024-11-03 19:56:28
chainsignal peptide:1-23
chain:24-92
peptide:24-33
peptide:37-92
checksum30A72221B076FA79
commentFUNCTION Stimulates the secretion of gonadotropins; it stimulates the secretion of both luteinizing and follicle-stimulating hormones.SUBCELLULAR LOCATION The precursor is cleaved by ACE, which removes the Gly-Lys-Arg peptide at the C-terminus, leading to mature hormone (PubMed:10336644, PubMed:7683654). The mature form of Gonadoliberin-1 is also cleaved and degraded by ACE (PubMed:2983326, PubMed:7683654).DISEASE The disease is caused by variants 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).PHARMACEUTICAL Available under the names Factrel (Ayerst Labs), Lutrepulse or Lutrelef (Ferring Pharmaceuticals) and Relisorm (Serono). Used in evaluating hypothalamic-pituitary gonadotropic function.MISCELLANEOUS The 3D-structure was determined for the synthetic analog Triptorelin.SIMILARITY Belongs to the GnRH family.ONLINE INFORMATION Gonadotropin-releasing hormone entry
descriptionrecommendedName: Progonadoliberin-1 alternativeName: Progonadoliberin I component recommendedName: Gonadoliberin-1 alternativeName: Gonadoliberin I alternativeName: Gonadorelin alternativeName: Gonadotropin-releasing hormone I shortName: GnRH-I alternativeName: Luliberin I alternativeName: Luteinizing hormone-releasing hormone I shortName: LH-RH I /component component recommendedName: GnRH-associated peptide 1 alternativeName: GnRH-associated peptide I /component
geneNameGNRH1
GNRH
GRH
LHRH
identifierP01148
isSequenceChangedFALSE
keyword3D-structure
Amidation
Cleavage on pair of basic residues
Direct protein sequencing
Disease variant
Hormone
Hypogonadotropic hypogonadism
Kallmann syndrome
Pharmaceutical
Proteomics identification
Pyrrolidone carboxylic acid
Reference proteome
Secreted
Signal
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
nameGNRH1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:9003552] ENSEMBL:ENSG00000147437 GNRH1 [Homo sapiens]
secondaryIdentifierGON1_HUMAN
A0AVP0
sequenceLength92
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:873934] GNRH1(24-33) [extracellular region] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:P01148 GNRH1 (55788)