Article
Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.
American journal of physiology. Gastrointestinal and liver physiology - 1 May 2004
Teckman Jeffrey H, An Jae-Koo, Blomenkamp Keith, Schmidt Bela, Perlmutter David
Abstract excerpt
Homozygous, PIZZ alpha(1)-antitrypsin (alpha(1)-AT) deficiency is associated with chronic liver disease and hepatocellular carcinoma resulting from the toxic effects of mutant alpha(1)-anti-trypsin Z (alpha(1)-ATZ) protein retained in the endoplasmic reticulum (ER) of hepatocytes. However, the exact mechanism(s) by which retention of this aggregated mutant protein leads to cellular injury are still unknown....
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