Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions.

Abstract excerpt

α-1-Antitrypsin (α1AT) is a serum glycoprotein synthesized in the liver. The majority of patients with α1AT deficiency liver disease are homozygous for the Z mutant of α1AT (called ZZ or 'PIZZ'). This mutant gene directs the synthesis of an abnormal protein which folds improperly during biogenesis. Most of these mutant Z protein molecules undergo proteolysis; however, some of the mutant protein accumulates in...

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