Biologic role(s) of the 25(R),26-hydroxycholesterol metabolic pathway.
Expression of the gene coding for the synthesis of 25(R), 26-hydroxycholesterol in many tissues and the finding that this sterol can be the sole pathway for the production of bile acids have led to a renewed interest in this metabolic pathway. A further impetus for exploring the normal biologic roles that are served by expression of the CYP27A1 gene is the knowledge that mutations in humans are associated with accelerated atherosclerosis and with severe neurologic impairment. The molecular mechanisms governing these phenotypic expressions are not known but in light of the traditional role of steroids as ligands for receptors that regulate gene expression it seems likely that the intermediates in this pathway modulate a number of enzymatic activities that remain to be elucidated.
Javitt
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Division of Hepatic Diseases, New York University School of Medicine, 550 First Avenue, New York, NY 10016, USA. norman.javitt@med.nyu.edu
D000818:Animals; D001161:Arteriosclerosis / Q000235:genetics; D001647:Bile Acids and Salts / Q000096:biosynthesis*; D053493:Cholestanetriol 26-Monooxygenase; D003577:Cytochrome P-450 Enzyme System / Q000235:genetics* / Q000502:physiology; D015971:Gene Expression Regulation, Enzymologic; D006801:Humans; D006888:Hydroxycholesterols / Q000378:metabolism*; D009154:Mutation; D009422:Nervous System Diseases / Q000235:genetics; D013237:Stereoisomerism; D013250:Steroid Hydroxylases / Q000235:genetics* / Q000502:physiology; D019294:Xanthomatosis, Cerebrotendinous / Q000235:genetics
D001647:Bile Acids and Salts; D006888:Hydroxycholesterols; C076996:27-hydroxycholesterol; D003577:Cytochrome P-450 Enzyme System; D013250:Steroid Hydroxylases; C506831:CYP27A1 protein, human; D053493:Cholestanetriol 26-Monooxygenase