Hepatocyte proteomes reveal the role of protein disulfide isomerase 4 in alpha 1-antitrypsin deficiency

Hepatocyte proteomes reveal the role of protein disulfide isomerase 4 in alpha 1-antitrypsin deficiency

Background & Aims: A single point mutation in the Z-variant of alpha 1-antitrypsin (Z-AAT) alone can lead to both a protein folding and trafficking defect, preventing its exit from the endoplasmic reticulum (ER), and the formation of aggregates that are retained as inclusions within the ER o...

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Bibliographic Details
Main Authors: Esra Karatas, Anne-Aurélie Raymond, Céline Leon, Jean-William Dupuy, Sylvaine Di-Tommaso, Nathalie Senant, Sophie Collardeau-Frachon, Mathias Ruiz, Alain Lachaux, Frédéric Saltel, Marion Bouchecareilh
Format: Article
Language:English
Published: Elsevier 2021-08-01
Series:JHEP Reports
Subjects:
Alpha 1-antitrypsin deficiency
Liver damage
Protein disulfide isomerase
PDIA4
Cysteamine
Treatment
Online Access:http://www.sciencedirect.com/science/article/pii/S2589555921000732

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http://www.sciencedirect.com/science/article/pii/S2589555921000732

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