Induced pluripotent stem cells model personalized variations in liver disease due to α(1)-antitrypsin deficiency

In the classical form of α(1)-antitrypsin deficiency (ATD), aberrant intracellular accumulation of misfolded mutant α(1)-antitrypsin Z (ATZ) in hepatocytes causes hepatic damage by a gain-of-function, “proteotoxic” mechanism. While some ATD patients develop severe liver disease that necessitates liv...

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Detalhes bibliográficos
Publicado no:Hepatology
Main Authors: Tafaleng, Edgar N., Chakraborty, Souvik, Han, Bing, Hale, Pamela, Wu, Wanquan, Soto-Gutierrez, Alejandro, Feghali-Bostwick, Carol A., Wilson, Andrew A., Kotton, Darrell N., Nagaya, Masaki, Strom, Stephen C., Chowdhury, Jayanta R., Stolz, Donna B., Perlmutter, David H., Fox, Ira J.
Formato: Artigo
Idioma:Inglês
Publicado em: 2015
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4482790/
https://ncbi.nlm.nih.gov/pubmed/25690322
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/hep.27753
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