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...
-д хадгалсан:
| -д хэвлэсэн: | Proc Natl Acad Sci U S A |
|---|---|
| Үндсэн зохиолчид: | , |
| Формат: | Artigo |
| Хэл сонгох: | Inglês |
| Хэвлэсэн: |
National Academy of Sciences
1980
|
| Нөхцлүүд: | |
| Онлайн хандалт: | 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 |
| Шошгууд: |
Шошго байхгүй, Энэхүү баримтыг шошголох эхний хүн болох!
|