Alpha‐1 antitrypsin Z protein (PiZ) increases hepatic fibrosis in a murine model of cholestasis
Ali Mencin, Ekihiro Seki, Yosuke Osawa, Yuzo Kodama, Samuele De Minicis, Michael Knowles, David A. Brenner – 29 October 2007 – Alpha‐1 antitrypsin (α1‐AT) deficiency is the most common genetic cause of liver disease in children. The homozygous α1‐ATZ mutation (PiZZ) results in significant liver disease in 10% of all affected patients. The α1‐ATZ mutation also may lead to worse liver injury in the setting of other liver diseases such as cystic fibrosis, nonalcoholic fatty liver disease, and hepatitis C.