Title : Efficacy of bezafibrate on fibroblasts of glutaric acidemia type II patients evaluated using an in vitro probe acylcarnitine assay.

Pub. Date : 2017 Jan

PMID : 27591119






1 Functional Relationships(s)
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1 RESULTS: The IVP assay showed that 100muM BEZ markedly reduced the level of palmitoylcarnitine (C16) in the neonatal-onset, infantile-onset, and myopathic forms of GA2, either increasing or maintaining a high level of acetylcarnitine (C2), which serves as an index of energy production via beta-oxidation. Palmitoylcarnitine electron transfer flavoprotein subunit alpha Homo sapiens