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Article

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

Molecular genetics and metabolism - 1 Mar 2016

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

Abstract excerpt

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 parameters to restore and maintain primary human hepatocyte biology and metabolism utilizing physiologically relevant...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 26740382
DOI: 10.1016/j.ymgme.2015.12.008
PMCID: PMC4852394

Genes

PCCA
PCCB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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