Trypsin 1

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PRSS1
Protein PRSS1 PDB 1trn.png
Available structures
PDB Human UniProt search: PDBe RCSB
Identifiers
Aliases PRSS1, TRP1, TRY1, TRY4, TRYP1, Trypsin 1, protease, serine 1
External IDs MGI: 3687012 HomoloGene: 134623 GeneCards: PRSS1
Targeted by Drug
camostat, nafamostat[1]
RNA expression pattern
PBB GE PRSS1 205869 at fs.png
More reference expression data
Orthologs
Species Human Mouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_002769

NM_001038996

RefSeq (protein)

NP_002760

n/a

Location (UCSC) Chr 7: 142.75 – 142.76 Mb Chr 6: 41.35 – 41.36 Mb
PubMed search [2] [3]
Wikidata
View/Edit Human View/Edit Mouse

Trypsin-1, also known as cationic trypsinogen, is a protein that in humans is encoded by the PRSS1 gene. Trypsin-1 is the main isoform of trypsinogen secreted by pancreas, the others are trypsin-2 (anionic trypsinogen), and trypsin-3 (meso-trypsinogen).

Function[edit]

This gene encodes a trypsinogen, which is a member of the trypsin family of serine proteases. This enzyme is secreted by the pancreas and cleaved to its active form in the small intestine. It is active on peptide linkages involving the carboxyl group of lysine or arginine. Mutations in this gene are associated with hereditary pancreatitis. This gene and several other trypsinogen genes are localized to the T cell receptor beta locus on chromosome 7.[4]

Clinical significance[edit]

Its malfunction acts in an autosomal dominant manner to cause pancreatitis. Many mutations that can lead to pancreatitis have been found.[5][6][7][8] An example is a mutation at Arg 117. Arg 117 is a trypsin-sensitive site which can be cleaved by another trypsin and becomes inactivated. This site may be a fail-safe mechanism by which trypsin, when activated within the pancreas, may become inactivated. Mutation at this cleavage site would result in a loss of control and permit autodigestion, causing pancreatitis.[9]

See also[edit]

References[edit]

  1. ^ "Drugs that physically interact with Protease, serine 1 view/edit references on wikidata". 
  2. ^ "Human PubMed Reference:". 
  3. ^ "Mouse PubMed Reference:". 
  4. ^ "Entrez Gene: PRSS1 protease, serine, 1 (trypsin 1)". 
  5. ^ Rebours V, Lévy P, Ruszniewski P (2011). "An overview of hereditary pancreatitiss". Digestive and Liver Disease. 44 (1): 8–15. doi:10.1016/j.dld.2011.08.003. PMID 21907651. 
  6. ^ Teich N, Mössner J, Keim V (1998). "Mutations of the cationic trypsinogen in hereditary pancreatitis". Hum. Mutat. 12 (1): 39–43. doi:10.1002/(SICI)1098-1004(1998)12:1<39::AID-HUMU6>3.0.CO;2-P. PMID 9633818. 
  7. ^ Chen JM, Ferec C (2000). "Molecular basis of hereditary pancreatitis.". Eur. J. Hum. Genet. 8 (7): 473–9. doi:10.1038/sj.ejhg.5200492. PMID 10909845. 
  8. ^ Gorry MC, Gabbaizedeh D, Furey W, et al. (1997). "Mutations in the cationic trypsinogen gene are associated with recurrent acute and chronic pancreatitis". Gastroenterology. 113 (4): 1063–8. doi:10.1053/gast.1997.v113.pm9322498. PMID 9322498. 
  9. ^ Whitcomb DC, Gorry MC, Preston RA, Furey W, Sossenheimer MJ, Ulrich CD, Martin SP, Gates LK, Amann ST, Toskes PP, Liddle R, McGrath K, Uomo G, Post JC, Ehrlich GD (1996). "Hereditary pancreatitis is caused by a mutation in the cationic trypsinogen gene". Nature Genetics. 14 (2): 141–5. doi:10.1038/ng1096-141. PMID 8841182. 

Further reading[edit]

External links[edit]

This article incorporates text from the United States National Library of Medicine, which is in the public domain.