The unfolded protein response to PI*Z alpha‐1 antitrypsin in human hepatocellular and murine models

The unfolded protein response to PI*Z alpha‐1 antitrypsin in human hepatocellular and murine models

Abstract Alpha‐1 antitrypsin (AAT) deficiency (AATD) is an inherited disease caused by mutations in the serpin family A member 1 (SERPINA1, also known as AAT) gene. The most common variant, PI*Z (Glu342Lys), causes accumulation of aberrantly folded AAT in the endoplasmic reticulum (ER) of hepatocyte...

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Main Authors: Yuanqing Lu, Liqun R. Wang, Jungnam Lee, Naweed S. Mohammad, Alek M. Aranyos, Calvin Gould, Nazli Khodayari, Regina A. Oshins, Craig G. Moneypenny, Mark L. Brantly
Format: Article
Language:English
Published: Wolters Kluwer Health/LWW 2022-09-01
Series:Hepatology Communications
Online Access:https://doi.org/10.1002/hep4.1997

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https://doi.org/10.1002/hep4.1997

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