Article
Mouse model of PRSS1 p.R122H-related hereditary pancreatitis highlights context-dependent effect of autolysis-site mutation.
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] - 1 Mar 2023
Jancsó Zsanett, Morales Granda Nataly C, Demcsák Alexandra, Sahin-Tóth Miklós
Abstract excerpt
Mutation p.R122H in human cationic trypsinogen (PRSS1) is the most frequently identified cause of hereditary pancreatitis. The mutation blocks protective degradation of trypsinogen by chymotrypsin C (CTRC), which involves an obligatory trypsin-mediated cleavage at Arg122. Previously, we found that C57BL/6N mice are naturally deficient in CTRC, and trypsinogen degradation is catalyzed by chymotrypsin B1 (CTRB1)....
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