In the small intestine, each isoform is cleaved by Enterokinase into its active form, Trypsin-1, Trypsin-2 and Trypsin-3, respectively. All trypsins are members of the serine protease trypsin family. The activated trypsins go on to activate other protease zymogens and play a role in the autoactivation of trypsinogens. This suggests an important role for trypsins in digestion. Mutations in the gene encoding Trypsin-1 that stimulate its activity are associated with autosomal dominant hereditary pancreatitis (HCP), also known as chronic pancreatitis (CP), a disease characterized by persistent, severe abdominal pain due to calcifications of the parenchyma, pancreatic stones, cysts and pancreatic head enlargement. Trypsin-3 is expressed in the brain in addition to the pancreas.
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