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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| 發表在: | Proc Natl Acad Sci U S A |
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| Principais autores: | , |
| 格式: | Artigo |
| 語言: | Inglês |
| 出版: |
National Academy of Sciences
1980
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| 主題: | |
| 在線閱讀: | 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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