Sterol 27-hydroxylase deficiency: a rare cause of xanthomas in normocholesterolemic humans. Trends Endocrinol Metab 2000;11:180-183.Ingemar Björkhem,Eran Leitersdorf.Sterol 27-hydroxylase Deficiency: A Rare Cause of Xanthomas in Normocholesterolemic Humans[J]. Trends in Endocrinology & Metabolism...
Cali JJ, Hsieh C-L, Francke U, Russell DW (1991) Mutations in the bile acid biosynthetic enzyme sterol 27-hydroxylase underlie cerebrotendinous xanthomatosis. J Biol Chem 266: 7779–7783 PubMed CAS Google Scholar Chang T-Y (1983) Mammalian HMG-CoA reductase and its regulation In: Boyer ...
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Matthias Johannes ReddehaseBundesforschungsanstalt für Viruskrankheiten der Tiere, TubingenJames Hilton CoxBundesforschungsanstalt für Viruskrankheiten der Tiere, TubingenUlrich Helmut KoszinowskiBundesforschungsanstalt für Viruskrankheiten der Tiere, TubingenAtherosclerosis Supplements...
Sterol 27-hydroxylase (CYP27) is a ubiquitously expressed enzyme involved in cholesterol metabolism. Notably, its deficiency causes a disease characterized by progressive neurologic impairment. With the final goal to understand the pathophysiological role of CYP27A1 in the CNS, we studied the ...
the 'alternative' or '27-hydroxylase' route to total bile acid synthesis, as demonstrated in cultured rat hepatocytes and in vivo in humans, we here evaluate the effects of various bile acids commonly found in bile of rats on the regulation of sterol 27-hydroxylase in cultured rat hepatocytes...
In man, hepatic mitochondrial sterol 27-hydroxylase and microsomal cholesterol 7α-hydroxylase initiate distinct pathways of bile acid biosynthesis from cholesterol, the "acidic" and "neutral" pathways, respectively. A similar acidic pathway in the rat has been hypothesized, but its quantitative importan...
Transfection of mutant cDNAs into cultured cells results in the synthesis of immunoreactive sterol 27-hydroxylase protein with greatly diminished enzyme activity. We have localized the CYP27 gene to the q33-qter interval of human chromosome 2, and to mouse chromosome 1, in agreement with the ...
In man, hepatic mitochondrial sterol 27-hydroxylase and microsomal cholesterol 7alpha-hydroxylase initiate distinct pathways of bile acid biosynthesis from cholesterol, the "acidic" and "neutral" pathways, respectively. A similar acidic pathway in the rat has been hypothesized, but its quantitative impor...
A method for preventing or reducing restenosis wherein a 27- hydroxycholesterol or a 25,26 and/or 27-aminocholesterol, or a sterol 27- hydroxylase stimulant, is administered in a restenosis preventing and/or reducing amount. The invention also includes a method for treating cancer using 27-hydro...