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Defect in biosynthesis of mitochondrial acetoacetyl-coenzyme A thiolase in cultured fibroblasts from a boy with 3-ketothiolase deficiency.

The etiology of 3-ketothiolase deficiency has been attributed to a defect of mitochondrial acetoacetyl-CoA thiolase because the acetoacetyl-CoA thiolase activity in related materials is not activated by K+, a property characteristic for this enzyme. We studied the enzyme protein and the biosynthesis...

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Bibliografske podrobnosti
Main Authors: Yamaguchi, S, Orii, T, Sakura, N, Miyazawa, S, Hashimoto, T
Format: Artigo
Jezik:Inglês
Izdano: 1988
Teme:
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC442530/
https://ncbi.nlm.nih.gov/pubmed/2893809
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