NorUDCA promotes degradation of α1-antitrypsin mutant Z protein by inducing autophagy through AMPK/ULK1 pathway

Author:

Tang Youcai,Blomenkamp Keith S.,Fickert Peter,Trauner Michael,Teckman Jeffrey H.ORCID

Funder

Alpha-1 Foundation

SSM Health Cardinal Glennon Children’s Foundation

Saint Louis University Liver Center

Publisher

Public Library of Science (PLoS)

Subject

Multidisciplinary

Reference51 articles.

1. Alpha-1-antitrypsin deficiency: diagnosis, pathophysiology, and management;JH Teckman;Curr. Gastroenterol. Rep,2006

2. In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA;PE Cruz;Lab. Invest,2007

3. Autophagic disposal of the aggregation-prone protein that causes liver inflammation and carcinogenesis in alpha-1-antitrypsin deficiency;DH Perlmutter;Cell Death Differ,2009

4. Hepatic fibrosis and carcinogenesis in alpha1-antitrypsin deficiency: a prototype for chronic tissue damage in gain-of-function disorders. Cold Spring Harb;DH Perlmutter;Perspect. Biol,2011

5. Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity;T Kamimoto;J. Biol. Chem,2006

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