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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Veröffentlicht in:Mol Genet Metab
Hauptverfasser: 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
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2015
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Article
Online Zugang: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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