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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 of he...

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Principais autores: 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: Artigo
Sprog:Inglês
Udgivet: Elsevier 2021-08-01
Serier:JHEP Reports
Fag:
Alpha 1-antitrypsin deficiency
Liver damage
Protein disulfide isomerase
PDIA4
Cysteamine
Treatment
Online adgang:http://www.sciencedirect.com/science/article/pii/S2589555921000732
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