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Gene Review

Cyp27a1  -  cytochrome P450, family 27, subfamily a,...

Mus musculus

Synonyms: 1300013A03Rik, 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 27-hydroxylase, Cyp27, Cytochrome P-450C27/25, Cytochrome P450 27, ...
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Disease relevance of Cyp27a1


High impact information on Cyp27a1


Chemical compound and disease context of Cyp27a1


Biological context of Cyp27a1


Anatomical context of Cyp27a1

  • In bile and feces of the cyp27(-/-) mice only traces of bile alcohols were found, and there was no cholestanol accumulation [12].
  • A compensatory 2.5-fold increase in whole body cholesterol synthesis occurs in Cyp27(-/-) mice, principally in liver, adrenal, small intestine, lung, and spleen [1].
  • RNA blotting experiments demonstrated sterol 27-hydroxylase mRNAs of approximately 1.8 to 2.2 kilobases in liver and fibroblast cells [10].
  • Introduction of the sterol 27-hydroxylase cDNA into Simian COS cells resulted in the expression of active enzyme capable of catalyzing multiple oxidation reactions (R-CH3----R-CH2OH----R-COOH) at carbon 27 of sterol intermediates of the bile acid synthesis pathway [10].
  • Agents that increase foam cell formation also down-regulate cholesterol 27-hydroxylase and ABCA1 expression [13].

Associations of Cyp27a1 with chemical compounds


Other interactions of Cyp27a1

  • Long-term (> 4 months) administration of P-407 to C57BL/6 mice appears to have no effect on the biologic activity of S27H and HMG-CoA reductase, but decreases the activity of C7alphaH [16].


  1. Disruption of the sterol 27-hydroxylase gene in mice results in hepatomegaly and hypertriglyceridemia. Reversal by cholic acid feeding. Repa, J.J., Lund, E.G., Horton, J.D., Leitersdorf, E., Russell, D.W., Dietschy, J.M., Turley, S.D. J. Biol. Chem. (2000) [Pubmed]
  2. Side chain hydroxylations in bile acid biosynthesis catalyzed by CYP3A are markedly up-regulated in Cyp27-/- mice but not in cerebrotendinous xanthomatosis. Honda, A., Salen, G., Matsuzaki, Y., Batta, A.K., Xu, G., Leitersdorf, E., Tint, G.S., Erickson, S.K., Tanaka, N., Shefer, S. J. Biol. Chem. (2001) [Pubmed]
  3. Sterol 27-hydroxylase deficiency: a rare cause of xanthomas in normocholesterolemic humans. Björkhem, I., Leitersdorf, E. Trends Endocrinol. Metab. (2000) [Pubmed]
  4. In vivo and in vitro regulation of sterol 27-hydroxylase in the liver during the acute phase response. potential role of hepatocyte nuclear factor-1. Memon, R.A., Moser, A.H., Shigenaga, J.K., Grunfeld, C., Feingold, K.R. J. Biol. Chem. (2001) [Pubmed]
  5. Hepatic cholesterol metabolism and resistance to dietary cholesterol in LXRbeta-deficient mice. Alberti, S., Schuster, G., Parini, P., Feltkamp, D., Diczfalusy, U., Rudling, M., Angelin, B., Björkhem, I., Pettersson, S., Gustafsson, J.A. J. Clin. Invest. (2001) [Pubmed]
  6. Identification of bile acid precursors as endogenous ligands for the nuclear xenobiotic pregnane X receptor. Goodwin, B., Gauthier, K.C., Umetani, M., Watson, M.A., Lochansky, M.I., Collins, J.L., Leitersdorf, E., Mangelsdorf, D.J., Kliewer, S.A., Repa, J.J. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  7. The Diet1 locus confers protection against hypercholesterolemia through enhanced bile acid metabolism. Phan, J., Pesaran, T., Davis, R.C., Reue, K. J. Biol. Chem. (2002) [Pubmed]
  8. Human sterol 27-hydroxylase (CYP27) overexpressor transgenic mouse model. Evidence against 27-hydroxycholesterol as a critical regulator of cholesterol homeostasis. Meir, K., Kitsberg, D., Alkalay, I., Szafer, F., Rosen, H., Shpitzen, S., Avi, L.B., Staels, B., Fievet, C., Meiner, V., Björkhem, I., Leitersdorf, E. J. Biol. Chem. (2002) [Pubmed]
  9. 7 alpha-hydroxylation of 27-hydroxycholesterol: biologic role in the regulation of cholesterol synthesis. Martin, K.O., Reiss, A.B., Lathe, R., Javitt, N.B. J. Lipid Res. (1997) [Pubmed]
  10. Characterization of human sterol 27-hydroxylase. A mitochondrial cytochrome P-450 that catalyzes multiple oxidation reaction in bile acid biosynthesis. Cali, J.J., Russell, D.W. J. Biol. Chem. (1991) [Pubmed]
  11. Fibrates suppress bile acid synthesis via peroxisome proliferator-activated receptor-alpha-mediated downregulation of cholesterol 7alpha-hydroxylase and sterol 27-hydroxylase expression. Post, S.M., Duez, H., Gervois, P.P., Staels, B., Kuipers, F., Princen, H.M. Arterioscler. Thromb. Vasc. Biol. (2001) [Pubmed]
  12. Markedly reduced bile acid synthesis but maintained levels of cholesterol and vitamin D metabolites in mice with disrupted sterol 27-hydroxylase gene. Rosen, H., Reshef, A., Maeda, N., Lippoldt, A., Shpizen, S., Triger, L., Eggertsen, G., Björkhem, I., Leitersdorf, E. J. Biol. Chem. (1998) [Pubmed]
  13. Adenosine A2A receptor occupancy stimulates expression of proteins involved in reverse cholesterol transport and inhibits foam cell formation in macrophages. Reiss, A.B., Rahman, M.M., Chan, E.S., Montesinos, M.C., Awadallah, N.W., Cronstein, B.N. J. Leukoc. Biol. (2004) [Pubmed]
  14. Over-expression of hepatic neutral cytosolic cholesteryl ester hydrolase in mice increases free cholesterol and reduces expression of HMG-CoAR, CYP27, and CYP7A1. Langston, T.B., Hylemon, P.B., Grogan, W.M. Lipids (2005) [Pubmed]
  15. Differences in hepatic levels of intermediates in bile acid biosynthesis between Cyp27(-/-) mice and CTX. Honda, A., Salen, G., Matsuzaki, Y., Batta, A.K., Xu, G., Leitersdorf, E., Tint, G.S., Erickson, S.K., Tanaka, N., Shefer, S. J. Lipid Res. (2001) [Pubmed]
  16. The P-407-induced murine model of dose-controlled hyperlipidemia and atherosclerosis: a review of findings to date. Johnston, T.P. J. Cardiovasc. Pharmacol. (2004) [Pubmed]
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