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Cerebrotendinous xanthomatosis: defective liver mitochondrial hydroxylation of chenodeoxycholic acid precursors.

Oxidation of the side chain of 5 beta-cholestane-3 alpha, 7 alpha-diol, 7 alpha-hydroxy-4-cholesten-3-one, and 5-cholestene-3 beta, 7 alpha-diol has been studied in subcellular fractions of liver from a patient with cerebrotendinous xanthomatosis (CTX) and a control subject. All intermediates were e...

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Bibliografiska uppgifter
Huvudupphov: H Oftebro, I Björkhem, F C Størmer, J I Pedersen
Materialtyp: Artigo
Språk:Inglês
Utgiven: Elsevier 1981-05-01
Serie:Journal of Lipid Research
Länkar:http://www.sciencedirect.com/science/article/pii/S0022227520373739
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