Both terminal misfolding and polymerisation contribute to disease-relevant phenotypic changes in cell models of alpha-1-antitrypsin deficiency-associated liver disease
Panayiota Stylianou, Kish R. Adoni, Mun Peak Nyon, Adam Cryar, Imran Haq, Jennifer A. Dickens, Charis‐Patricia Segeritz, Graeme C. Smith, Adriana Ordóñez, James A. Irving, David A. Lomas, Kyra-Mae Leighton, Jessica Beasley, Aleck W.E. Jones, S. Tamir Rashid, , Bibek Gooptu
This study shows that both terminal misfolding and polymerisation of Z 1-antitrypsin contribute to liver disease phenotypes, with terminal misfolding driving pro-fibrotic responses and metabolic changes.
bioRxiv · Sep 30, 2025 · PreprintCell Biology