Article
Autophagy induced by exogenous bile acids is therapeutic in a model of α-1-AT deficiency liver disease.
American journal of physiology. Gastrointestinal and liver physiology - 1 Jul 2016
Tang Youcai, Fickert Peter, Trauner Michael, Marcus Nancy, Blomenkamp Keith, Teckman Jeffrey
Abstract excerpt
The bile acid nor-ursodeoxycholic acid (norUDCA) has many biological actions, including antiapoptotic effects. Homozygous PIZZ α-1-antitrypsin (A1AT)-deficient humans are known to be at risk for liver disease, cirrhosis, and liver cancer as a result of the accumulation of the toxic, A1AT mutant Z protein within hepatocytes. This accumulation triggers cell death in the hepatocytes with the largest mutant Z-protein...
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