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Biotin activation of carboxylase activity in cultured fibroblasts from a child with a combined carboxylase defect

✍ Scribed by K. Bartlett; D. Gompertz

Book ID
115822207
Publisher
Elsevier Science
Year
1978
Tongue
English
Weight
199 KB
Volume
84
Category
Article
ISSN
0009-8981

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Evaluation of biotin responsiveness in cultured fibroblasts from patients with propionic acidemia: Absence of response by structurally altered carboxylases
✍ Barry Wolf πŸ“‚ Article πŸ“… 1979 πŸ› Springer 🌐 English βš– 288 KB

We have demonstrated that, although propionyl CoA carboxylase (PCC) activity is deficient in fibroblast extracts from PCC-deficient patients belonging to the two major and two minor genetic complementation groups, the activity of another biotin-dependent carboxylase, beta-methylcrotonyl CoA carboxyl