Additional pathways of sterol metabolism: Evidence from analysis of Cyp27a1−/− mouse brain and plasma

Cytochrome P450 (CYP) 27A1 is a key enzyme in both the acidic and neutral pathways of bile acid biosynthesis accepting cholesterol and ring-hydroxylated sterols as substrates introducing a (25R)26-hydroxy and ultimately a (25R)26-acid group to the sterol side-chain. In human, mutations in the CYP27A...

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Veröffentlicht in:	Biochimica et biophysica acta. Molecular and cell biology of lipids 2019-02, Vol.1864 (2), p.191-211
Hauptverfasser:	Griffiths, William J., Crick, Peter J., Meljon, Anna, Theofilopoulos, Spyridon, Abdel-Khalik, Jonas, Yutuc, Eylan, Parker, Josie E., Kelly, Diane E., Kelly, Steven L., Arenas, Ernest, Wang, Yuqin
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals bile acids Bile Acids and Salts - biosynthesis biosynthesis Brain Brain - metabolism Cerebrotendinous xanthomatosis Cholestanetriol 26-Monooxygenase - genetics Cholestanetriol 26-Monooxygenase - physiology Cholestenes - metabolism Cholestenoic acid Cholesterol - metabolism Chromatography, Liquid CYP27A1 cytochrome P-450 Cytochrome P-450 Enzyme System - metabolism enzyme activity enzymes genes humans Hydroxylation knockout mutants ligands Lipid Metabolism - physiology liquid chromatography liver Liver X Receptors - metabolism Male Mass spectrometry metabolites Mice Mice, Inbred C57BL Mice, Knockout motor neurons mutation Oxysterol oxysterols Oxysterols - metabolism patients phenotype Pregnane X Receptor - metabolism pregnanes stereochemistry Sterols - metabolism Tandem Mass Spectrometry Xanthomatosis, Cerebrotendinous
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