Recapitulation of metabolic defects in a model of propionic acidemia using patient-derived primary hepatocytes

BACKGROUND: Propionic acidemia (PA) is a disorder of intermediary metabolism with defects in the alpha or beta subunits of propionyl CoA carboxylase (PCCA and PCCB respectively) enzyme. We previously described a liver culture system that uses liver-derived hemodynamic blood flow and transport parame...

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Detalhes bibliográficos
Publicado no:Mol Genet Metab
Main Authors: Chapman, Kimberly A., Collado, Maria S., Figler, Robert A., Hoang, Stephen A., Armstrong, Allison J., Cui, Wanxing, Purdy, Michael, Simmers, Michael B., Yazigi, Nada A., Summar, Marshall L., Wamhoff, Brian R., Dash, Ajit
Formato: Artigo
Idioma:Inglês
Publicado em: 2015
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4852394/
https://ncbi.nlm.nih.gov/pubmed/26740382
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2015.12.008
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