Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.

To study the enzymatic basis of isovaleric acidemia, we have developed assay methods for isovaleryl-CoA and butyryl-CoA dehydrogenases that measure the amount of tritium released from the respective [2,3-3H]acyl CoAs. Because assay of these enzymes in human fibroblast homogenates was subject to inte...

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Veröffentlicht in:	Proc Natl Acad Sci U S A
Hauptverfasser:	Rhead, W J, Tanaka, K
Format:	Artigo
Sprache:	Inglês
Veröffentlicht:	National Academy of Sciences 1980
Schlagworte:	Research Article
Online-Zugang:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC348317/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/6928646/ https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.77.1.580
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