Trypsin-1, also known ascationic trypsinogen, is aprotein that in humans is encoded by thePRSS1gene. Trypsin-1 is the mainisoform oftrypsinogen secreted by pancreas, the others aretrypsin-2 (anionic trypsinogen), andtrypsin-3 (meso-trypsinogen).
This gene encodes a trypsinogen, which is a member of thetrypsin family ofserine 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 thecarboxyl group oflysine orarginine. Mutations in this gene are associated with hereditarypancreatitis. This gene and several other trypsinogen genes are localized to the T cell receptor beta locus on chromosome 7.[5]
Its malfunction acts in an autosomal dominant manner to cause pancreatitis. Many mutations that can lead to pancreatitis have been found.[6][7][8][9] 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.[10]
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