hiPSC hepatocyte model demonstrates the role of unfolded protein response and inflammatory networks in α1-antitrypsin deficiency

α1-antitrypsin (A1AT) is a 52 kDa protein encoded by the SERPINA1 gene synthesized primarily by hepatocytes [1]. Secreted into the blood stream, it acts to control the function of neutrophil elastase, particularly in the lung [2]. A1AT also exerts anti-apoptotic and anti-inflammatory properties dur ing inflammation and hepatic injury. Most people carry the wildtype M allele, while the rarer Z variant (found in 1-3% of the population), is associated with the most common and severe form of clinically significant A1AT deficiency (A1ATD) [3].

Source: Journal of Hepatology - June 4, 2018 Category: Gastroenterology Authors: Charis-Patricia Segeritz, Sheikh Tamir Rashid, Miguel Cardoso de Brito, Maria Serra Paola, Adriana Ordonez, Carola Maria Morell, Joseph E. Kaserman, Pedro Madrigal, Nicholas Hannan, Laurent Gatto, Lu Tan, Andrew A. Wilson, Kathryn Lilley, Stefan J. Marcin Source Type: research