Metabolism of the cholestanol precursor cholesta-4,6-dien-3-one in different tissues
Author:
Publisher
Elsevier BV
Subject
Endocrinology,Biochemistry,Biophysics
Reference13 articles.
1. Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid.
2. Role of the 26-hydroxylase in the biosynthesis of bile acids in the normal state and in cerebrotendinous xanthomatosis. An in vivo study.
3. Assay of intermediates in bile acid biosynthesis using isotope dilution–mass spectrometry: hepatic levels in the normal state and in cerebrotendinous xanthomatosis.
4. A novel pathway for biosynthesis of cholestanol with 7 alpha-hydroxylated C27-steroids as intermediates, and its importance for the accumulation of cholestanol in cerebrotendinous xanthomatosis.
5. Accumulation of 7α-hydroxy-4-cholesten-3-one and cholesta-4,6-dien-3-one in patients with cerebrotendinous xanthomatosis: Effect of treatment with chenodeoxycholic acid
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1. Cholesterol metabolism in mice models of genetic hypercholesterolemia;Journal of Physiology and Biochemistry;2020-06-17
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. Biodegradation of cytotoxic 7-Ketocholesterol by Pseudomonas aeruginosa PseA;Bioresource Technology;2016-08
4. Profiling sterols in cerebrotendinous xanthomatosis: Utility of Girard derivatization and high resolution exact mass LC–ESI-MSn analysis;Journal of Chromatography B;2011-05
5. On the mechanism of cerebral accumulation of cholestanol in patients with cerebrotendinous xanthomatosis;Journal of Lipid Research;2007-05
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