The unfolded protein response to PI*Z alpha‐1 antitrypsin in human hepatocellular and murine models

Alpha‐1 antitrypsin (AAT) deficiency (AATD) is an inherited disease caused by mutations in the serpin family A member 1 (SERPINA1, also known as AAT) gene. The most common variant, PI*Z (Glu342Lys), causes accumulation of aberrantly folded AAT in the endoplasmic reticulum (ER) of hepatocytes that is...

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Detalles Bibliográficos
Autores principales: Lu, Yuanqing, Wang, Liqun R., Lee, Jungnam, Mohammad, Naweed S., Aranyos, Alek M., Gould, Calvin, Khodayari, Nazli, Oshins, Regina A., Moneypenny, Craig G., Brantly, Mark L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
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Original Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9426387/
https://www.ncbi.nlm.nih.gov/pubmed/35621045
http://dx.doi.org/10.1002/hep4.1997

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