Role of the 26-hydroxylase in the biosynthesis of bile acids in the normal state and in cerebrotendinous xanthomatosis. An in vivo study.

On the basis of different in vitro studies, we have previously suggested that the basic metabolic defect in the rare inherited disease cerebrotendinous xanthomatosis (CTX) is a lack of a hepatic mitochondrial C27-steroid 26-hydroxylase, involved in the normal biosynthesis of bile acids (1980. J. Cli...

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Hauptverfasser: Björkhem, I, Fausa, O, Hopen, G, Oftebro, H, Pedersen, J I, Skrede, S
Format: Artigo
Sprache:Inglês
Veröffentlicht: 1983
Schlagworte:
Research Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC436846/
https://ncbi.nlm.nih.gov/pubmed/6848555
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