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Human iPSC-hepatocyte modeling of alpha-1 antitrypsin heterozygosity reveals metabolic dysregulation and cellular heterogeneity

Individuals homozygous for the “Z” mutation in alpha-1 antitrypsin deficiency are known to be at increased risk for liver disease. It has also become clear that some degree of risk is similarly conferred by the heterozygous state. A lack of model systems that recapitulate heterozygosity in human hep...

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Detalles Bibliográficos
Autores principales: Kaserman, Joseph E., Werder, Rhiannon B., Wang, Feiya, Matte, Taylor, Higgins, Michelle I., Dodge, Mark, Lindstrom-Vautrin, Jonathan, Bawa, Pushpinder, Hinds, Anne, Bullitt, Esther, Caballero, Ignacio S., Shi, Xu, Gerszten, Robert E., Brunetti-Pierri, Nicola, Liesa, Marc, Villacorta-Martin, Carlos, Hollenberg, Anthony N., Kotton, Darrell N., Wilson, Andrew A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9780780/
https://www.ncbi.nlm.nih.gov/pubmed/36476855
http://dx.doi.org/10.1016/j.celrep.2022.111775