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

J Li, F Moretti, T Hidvegi, S Sviben… - Cellular and Molecular …, 2024 - Elsevier
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 …