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Biosynthesis and maturation of peroxisomal beta-oxidation enzymes in fibroblasts in relation to the Zellweger syndrome and infantile Refsum disease.

The biosynthesis of the peroxisomal enzymes acyl-CoA oxidase, 3-oxoacyl-CoA thiolase (acetyl-CoA acyl-transferase, EC 2.3.1.16), and catalase (EC 1.11.1.6) was studied in cultured skin fibroblasts from a control subject and from patients with Zellweger syndrome and the infantile form of Refsum disea...

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Udgivet i:Proc Natl Acad Sci U S A
Principais autores: Schram, A W, Strijland, A, Hashimoto, T, Wanders, R J, Schutgens, R B, van den Bosch, H, Tager, J M
Format: Artigo
Sprog:Inglês
Udgivet: National Academy of Sciences 1986
Fag:
Research Article
Online adgang:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC386458/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/2426710/
https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.83.16.6156
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