[HTML][HTML] Intracellular inclusions containing mutant α1-antitrypsin Z are propagated in the absence of autophagic activity
T Kamimoto, S Shoji, T Hidvegi, N Mizushima… - Journal of Biological …, 2006 - ASBMB
Mutant α 1-antitrypsin Z (α 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 α 1-
antitrypsin (α 1-AT) deficiency. Previous studies have suggested that efficient intracellular
degradation of α 1-ATZ is correlated with protection from liver disease in α 1-AT deficiency
and that the ubiquitin-proteasome system accounts for a major route, but not the sole route …
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 α 1-
antitrypsin (α 1-AT) deficiency. Previous studies have suggested that efficient intracellular
degradation of α 1-ATZ is correlated with protection from liver disease in α 1-AT deficiency
and that the ubiquitin-proteasome system accounts for a major route, but not the sole route …