hiPSC hepatocyte model demonstrates the role of unfolded protein response and inflammatory networks in α(1)-antitrypsin deficiency

BACKGROUND & AIMS: α(1)-Antitrypsin deficiency (A1ATD) is an autosomal recessive disorder caused by mutations in the SERPINA1 gene. Individuals with the Z variant (Gly342Lys) retain polymerised protein in the endoplasmic reticulum (ER) of their hepatocytes, predisposing them to liver disease. Th...

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Detalles Bibliográficos
Autores principales: Segeritz, Charis-Patricia, Rashid, Sheikh Tamir, de Brito, Miguel Cardoso, Serra, Maria Paola, Ordonez, Adriana, Morell, Carola Maria, Kaserman, Joseph E., Madrigal, Pedro, Hannan, Nicholas R.F., Gatto, Laurent, Tan, Lu, Wilson, Andrew A., Lilley, Kathryn, Marciniak, Stefan J., Gooptu, Bibek, Lomas, David A., Vallier, Ludovic
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6562205/
https://www.ncbi.nlm.nih.gov/pubmed/29879455
http://dx.doi.org/10.1016/j.jhep.2018.05.028

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