Hereditary pancreatitis caused by a three amino-acid insertion within the activation peptide of human cationic trypsinogen (PRSS1)

Abstract

s / Pancreatology 12 (2012) 502–597 544 Results: Absence of PMN-elastase led to amilder course of pancreatitis. Interestingly, neutrophils from PMN knock-out mice had significantly higher endogenous MPO activities. Addition of PMN-depleted splenocytes to CCK-stimulated acini induced less intracellular trypsinogen activation than splenocytes from wildtype animals. Along the same line, purified PMN-elastase induced intracellular trypsin activation in isolated acini whereas pancreatic elastase did not. Conclusion: The presence or absence of PMN-elastase affects the disease severity in acute experimental pancreatitis. PNM-elastase is not only involved in the transmigration of leukocytes into the pancreatic tissue during pancreatitis but also has a direct effect on intra-acinar cell trypsinogen activation.