A pancreatitis-associated chymotrypsinogen C mutant elicits endoplasmic reticulum stress and apoptosis
R Szmola, M Sahin-Tóth · Zeitschrift für Gastroenterologie · 2009 · Heft 05
Bibliografische Angaben
Zusammenfassung
Background and Aims: We recently identified chymotrypsinogen C (CTRC) as a novel susceptibility gene for chronic pancreatitis in humans (Nat Genet 2008; 40:78–82). Pancreatitis-associated CTRC mutants exhibit diminished secretion due to intracellular retention and degradation. We hypothesized that mutation-induced CTRC retention may result in endoplasmic reticulum (ER) stress and activate the unfolded protein response (UPR). Since prolonged activation of the UPR can lead to apoptotic cell death, a direct link may be established between CTRC mutants and acinar cell death in chronic pancreatitis. The aim of the pr…