hiPSC hepatocyte model demonstrates the role of unfolded protein response and inflammatory networks in α1-antitrypsin deficiency
Published version
Peer-reviewed
Repository URI
Repository DOI
Change log
Authors
Vallier, Ludovic https://orcid.org/0000-0002-3848-2602
Segeritz, Charis
Rashid, Sheiikh
Cardoso De Brito, miguel
Morell, Carola
Abstract
This study compared the gene expression and protein profiles of healthy liver cells and those affected by the inherited disease α1-antitrypsin deficiency. This approach identified specific factors primarily present in diseased samples which could provide new targets for drug development. This study also demonstrates the interest of using hepatic cells generated from human induced pluripotent stem cells to model liver disease in vitro for uncovering new mechanisms with clinical relevance.
Description
Keywords
StemCellInstitute, Hepatocyte, inherited liver disease, human-induced pluripotent stem cell, α1-Antitrypsin deficienc, inflammation
Journal Title
Journal of Hepatology
Conference Name
Journal ISSN
1600-0641
1600-0641
1600-0641
Volume Title
69
Publisher
Elsevier
Publisher DOI
Sponsorship
Medical Research Council (G1002610)
Medical Research Council (G0601840)
Alpha One Foundation (unknown)
Medical Research Council (MC_PC_12009)
National Centre for the Replacement Refinement and Reduction of Animals in Research (NC/N001540/1)
National Centre for the Replacement Refinement and Reduction of Animals in Research (NC/R001987/1)
European Research Council (741707)
Medical Research Council (MR/R009120/1)
Medical Research Council (G0601840)
Alpha One Foundation (unknown)
Medical Research Council (MC_PC_12009)
National Centre for the Replacement Refinement and Reduction of Animals in Research (NC/N001540/1)
National Centre for the Replacement Refinement and Reduction of Animals in Research (NC/R001987/1)
European Research Council (741707)
Medical Research Council (MR/R009120/1)
CPS is funded through a Children’s Liver Disease Foundation (CLDF)
studentship. DAL is funded by the Medical Research Council, Wellcome Trust, GlaxoSmithKline, the Rosetrees Trust, EPSRC and UCLH NIHR Biomedical Research Centre. LV, PM, MCB, NH are funded by ERC Relieve-IMDs and ERC advanced grant New-Chol, Cambridge University Hospitals National Institute for Health Research Biomedical Research Center, and the core support grant from the Wellcome Trust and Medical Research Council to the Wellcome Trust – Medical Research Council Cambridge Stem Cell Institute. STR is funded by an MRC Clinician Scientist Fellowship award.