Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1‐antitrypsin deficiency
Adriana Ordóñez, Erik L. Snapp, Lu Tan, Elena Miranda, Stefan J. Marciniak, David A. Lomas – 29 November 2012 – Point mutants of alpha1‐antitrypsin (α1AT) form ordered polymers that are retained as inclusions within the endoplasmic reticulum (ER) of hepatocytes in association with neonatal hepatitis, cirrhosis, and hepatocellular carcinoma. These inclusions cause cell damage and predispose to ER stress in the absence of the classical unfolded protein response (UPR).