Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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Chemical Compound Review:

AC1L18KF 10,13-dimethyl-17-(6- methylheptan-2-yl)-2...

Synonyms: cholestane-3,7,12-triol

Oftebro, H. et al., Okuda, K. et al., Ohyama, Y. et al., Dueland, S. et al.

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Disease relevance of 5-BETA-CHOLESTANE-3-ALPHA7-ALPHA12-ALP

Oxidation of side chain of 5 beta-cholestane-3 alpha,7 alpha,12 alpha-triol was studied in a patient with cerebrotendinous xanthomatosis (CTX) and in control subjects, using various subcellular fractions of liver homogenate and a method based on isotope dilution-mass spectrometry [1].

High impact information on 5-BETA-CHOLESTANE-3-ALPHA7-ALPHA12-ALP

5 beta-Cholestane-3 alpha,7 alpha,12 alpha-triol 27-hydroxylase (5 beta-cholestane-3 alpha,7 alpha,12 alpha-triol, NADPH:oxygen oxidoreductase (26-hydroxylating), EC 1.14.13.15) was purified from female rat liver mitochondria based on its catalytic activity [2].
26-hydroxylation of 5 beta-cholestane-3 alpha,7 alpha,12 alpha-triol by isolated nonparenchymal cells and hepatocytes from rat liver [3].
From these results it was concluded that 5 beta-cholestane-3 alpha,7 alpha,12 alpha-triol 27-hydroxylation and 1 alpha-hydroxyvitamin D3 25-hydroxylation are catalyzed by a common enzyme in rat liver mitochondria [4].

References

Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid. Oftebro, H., Björkhem, I., Skrede, S., Schreiner, A., Pederson, J.I. J. Clin. Invest. (1980) [Pubmed]
Purification and characterization of 5 beta-cholestane-3 alpha,7 alpha,12 alpha-triol 27-hydroxylase from female rat liver mitochondria. Okuda, K., Masumoto, O., Ohyama, Y. J. Biol. Chem. (1988) [Pubmed]
26-hydroxylation of 5 beta-cholestane-3 alpha,7 alpha,12 alpha-triol by isolated nonparenchymal cells and hepatocytes from rat liver. Dueland, S., Pedersen, J.I., Drevon, C.A., Björkhem, I. J. Lipid Res. (1982) [Pubmed]
Multi-functional property of rat liver mitochondrial cytochrome P-450. Ohyama, Y., Masumoto, O., Usui, E., Okuda, K. J. Biochem. (1991) [Pubmed]

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