Hepatic 7 alpha-dehydroxylation of bile acid intermediates, and its significance for the pathogenesis of cerebrotendinous xanthomatosis.
Author:
Publisher
Elsevier BV
Subject
Cell Biology,Endocrinology,Biochemistry
Reference31 articles.
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2. The metabolism of sterols and bile acids in cerebrotendinous xanthomatosis;Salen,1976
3. Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylase required for normal biosynthesis of cholic acid;Oftebro;J. Clin. Invest.,1980
4. Role of the 26-hydroxylase in the biosynthesis of bile acids in the normal state and in CTX. An in vivo study;Björkhem;J. Clin. Invest.,1983
5. On the metabolism of Δ4, 5-cholestenone;Anker;J. Biol. Chem.,1949
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2. Cytochrome P450 27A1 Deficiency and Regional Differences in Brain Sterol Metabolism Cause Preferential Cholestanol Accumulation in the Cerebellum;Journal of Biological Chemistry;2017-03
3. Profiling sterols in cerebrotendinous xanthomatosis: Utility of Girard derivatization and high resolution exact mass LC–ESI-MSn analysis;Journal of Chromatography B;2011-05
4. ESI-MS/MS quantification of 7α-hydroxy-4-cholesten-3-one facilitates rapid, convenient diagnostic testing for cerebrotendinous xanthomatosis;Clinica Chimica Acta;2010-01
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