Controlling autophagy: A new concept for clearing liver disease
Frank Tacke, Christian Trautwein – 12 January 2011 – In the classical form of alpha1‐antitrypsin (AT) deficiency, a point mutation in AT alters the folding of a liver‐derived secretory glycoprotein and renders it aggregation‐prone. In addition to decreased serum concentrations of AT, the disorder is characterized by accumulation of the mutant alpha1‐antitrypsin Z (ATZ) variant inside cells, causing hepatic fibrosis and/or carcinogenesis by a gain‐of‐toxic function mechanism. The proteasomal and autophagic pathways are known to mediate degradation of ATZ.