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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
1981-05-01
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| Series: | Journal of Lipid Research |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0022227520373739 |