Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity.

Abstract excerpt

Mutant alpha(1)-antitrypsin Z (alpha(1)-ATZ) protein, which has a tendency to form aggregated polymers as it accumulates within the endoplasmic reticulum of the liver cells, is associated with the development of chronic liver injury and hepatocellular carcinoma in hereditary alpha(1)-antitrypsin (alpha(1)-AT) deficiency. Previous studies have suggested that efficient intracellular degradation of alpha(1)-ATZ is...

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