Article
Preventing and reversing the cellular consequences of Z alpha-1 antitrypsin accumulation by targeting s4A.
Journal of hepatology - 1 Jul 2012
Alam Sam, Wang Jicun, Janciauskiene Sabina, Mahadeva Ravi
Abstract excerpt
BACKGROUND & AIMS: The Z variant (Glu342Lys) of α(1)-antitrypsin (AT) polymerizes and accumulates in the hepatocyte endoplasmic reticulum (ER) predisposing to neonatal hepatitis and liver cirrhosis. The resultant secretory defect leaves the lungs vulnerable to elastolysis and early-onset emphysema. Our aim in this study was to evaluate the effect of targeting strand 4a (s4A) as a strategy to inhibit...
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