Multiple Genes Core to ERAD, Macroautophagy and Lysosomal Degradation Pathways Participate in the Proteostasis Response in α1-Antitrypsin DeficiencySummary

Background & Aims: In the classic form of α1-antitrypsin deficiency (ATD), the misfolded α1-antitrypsin Z (ATZ) variant accumulates in the endoplasmic reticulum (ER) of liver cells. A gain-of-function proteotoxic mechanism is responsible for chronic liver disease in a subgroup of homozygotes. Pr...

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Principais autores:	Jie Li, Francesca Moretti, Tunda Hidvegi, Sanja Sviben, James A.J. Fitzpatrick, Hemalatha Sundaramoorthi, Stephen C. Pak, Gary A. Silverman, Britta Knapp, Ireos Filipuzzi, John Alford, John Reece-Hoyes, Florian Nigsch, Leon O. Murphy, Beat Nyfeler, David H. Perlmutter
פורמט:	Artigo
שפה:	Inglês
יצא לאור:	Elsevier 2024-01-01
סדרה:	Cellular and Molecular Gastroenterology and Hepatology
נושאים:	Autophagy Proteasome Aggregation-Prone Proteins α1-Antitrypsin Deficiency Liver Disease
גישה מקוונת:	http://www.sciencedirect.com/science/article/pii/S2352345X24000341
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