Ezetimibe normalizes metabolic defects in mice lacking ABCG5 and ABCG8

Journal of Lipid Research

Volumn 46, Issue 8, 2005, Pages 1739-1744

  Yu, Liqing ^a,d   Von Bergmann, Klaus ^c   Lütjohann, Dieter ^c   Hobbs, Helen H ^a,b   Cohen, Jonathan C ^a,b  

^a UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

^b UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

^c UNIVERSITY OF BONN   (Germany)

^d WAKE FOREST SCHOOL OF MEDICINE   (United States)

Author keywords

ATP binding cassette transporter G5; ATP binding cassette transporter G8; Bile; Cholesterol; Sitosterolemia

Indexed keywords

ABC TRANSPORTER; ABC TRANSPORTER G5; ABC TRANSPORTER G8; CHOLESTEROL; EZETIMIBE; HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE; HYDROXYMETHYLGLUTARYL COENZYME A SYNTHASE; MESSENGER RNA; PHYTOSTEROL; SITOSTEROL; UNCLASSIFIED DRUG;

ANIMAL MODEL; ARTICLE; BILE; BIOACCUMULATION; CHOLESTEROL BLOOD LEVEL; CONTROLLED STUDY; DIETARY INTAKE; DRUG EFFECT; LIVER METABOLISM; METABOLIC DISORDER; MOUSE; NONHUMAN; PHARMACOLOGICAL BLOCKING; PRIORITY JOURNAL; PROTEIN DEFICIENCY; STEROL METABOLISM;

ABSORPTION; ANIMALS; ATP-BINDING CASSETTE TRANSPORTERS; AZETIDINES; CHOLESTEROL; LIPOPROTEINS; LIVER; MICE; MICE, KNOCKOUT; PHYTOSTEROLS; SITOSTEROLS; STEROLS;

EID: 23244436716     PISSN: 00222275     EISSN: None     Source Type: Journal    
DOI: 10.1194/jlr.M500124-JLR200     Document Type: Article

References (33)

1. Berge, K. E., H. Tian, G. A. Graf, L. Yu, N. V. Grishin, J. Schultz, P. Kwiterovich, B. Shan, R. Barnes, and H. H. Hobbs. 2000. Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters. Science. 290: 1771-1775.
2. Lee, M. H., K. Lu, S. Hazard, H. Yu, S. Shulenin, H. Hidaka, H. Kojima, R. Allikmets, N. Sakuma, R. Pegoraro, et al. 2001. Identification of a gene, ABCG5, important in the regulation of dietary cholesterol absorption. Nat. Genet. 27: 79-83.
3. Bhattacharyya, A. K., and W. E. Connor. 1974. Beta-sitosterolemia and xanthomatosis. A newly described lipid storage disease in two sisters. J. Clin. Invest. 53: 1033-1043.
4. Miettinen, T. A. 1980. Phytosterolaemia, xanthomatosis and pre-mature atherosclerotic arterial disease: a case with high plant sterol absorption, impaired sterol elimination and low cholesterol synthesis. Eur. J. Clin. Invest. 10: 27-35.
5. Salen, G., V. Shore, G. S. Tint, T. Forte, S. Shefer, I. Horak, E. Horak, B. Dayal, L. Nguyen, A. K. Batta, et al. 1989. Increased sitosterol absorption, decreased removal, and expanded body pools compensate for reduced cholesterol synthesis in sitosterolemia with xanthomatosis. J. Lipid Res. 30: 1319-1330.
6. Lutjohann, D., I. Bjorkhem, U. F. Beil, and K. von Bergmann. 1995. Sterol absorption and sterol balance in phytosterolemia evaluated by deuterium-labeled sterols: effect of sitostanol treatment. J. Lipid Res. 36: 1763-1773.
7. Morganroth, J., R. I. Levy, A. E. McMahon, and A. M. Gotto, Jr. 1974. Pseudohomozygous type II hyperlipoproteinemia. J. Pediatr. 85: 639-643.
8. Belamarich, P. F., R. J. Deckelbaum, T. J. Stare, B. E. Dobrin, G. S. Tint, and G. Salen. 1990. Response to diet and cholestyramine in a patient with sitosterolemia. Pediatrics. 86: 977-981.
9. Salen, G., I. Horak, M. Rothkopf, J. L. Cohen, J. Speck, G. S. Tint, V. Shore, B. Dayal, T. Chen, and S. Shefer. 1985. Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis. J. Lipid Res. 26: 1126-1133.
10. Kolovou, G., V. Voudris, E. Drogari, G. Palatianos, and D. V. Cokkinos. 1996. Coronary bypass grafts in a young girl with sitosterolemia. Eur. Heart J. 17: 965-966.
11. Salen, G., S. Shefer, L. Nguyen, G. C. Ness, G. S. Tint, and V. Shore. 1992. Sitosterolemia. J. Lipid Res. 33: 945-955.
12. Bjorkhem, I., K. Boberg, and E. Leitersdorf. 2001. Inborn errors in bile acid biosynthesis and storage of sterols other than cholesterol. In The Metabolic and Molecular Bases of Inherited Disease. C. Scriver, A. Beaudet, W. Sly, and D. Valle, editors. New York, McGraw-Hill. 2961-2988.
13. Mymin, D., J. Wang, J. Frohlich, and R. A. Hegele. 2003. Image in cardiovascular medicine. Aortic xanthomatosis with coronary ostial occlusion in a child homozygous for a nonsense mutation in ABCG8. Circulation. 107: 791.
14. Parsons, H. G., R. Jamal, B. Baylis, V. C. Dias, and D. Roncari. 1995. A marked and sustained reduction in LDL sterols by diet and cholestyramine in beta-sitosterolemia. Clin. Invest. Med. 18: 389-400.
15. Nguyen, L. B., M. Cobb, S. Shefer, G. Salen, G. C. Ness, and G. S. Tint. 1991. Regulation of cholesterol biosynthesis in sitosterolemia: effects of lovastatin, cholestyramine, and dietary sterol restriction. J. Lipid Res. 32: 1941-1948.
16. Cobb, M. M., G. Salen, G. S. Tint, J. Greenspan, and L. B. Nguyen. 1996. Sitosterolemia: opposing effects of cholestyramine and lovastatin on plasma sterol levels in a homozygous girl and her heterozygous father. Metabolism. 45: 673-679.
17. Gregg, R. E., W. E. Connor, D. S. Lin, and H. B. Brewer, Jr. 1986. Abnormal metabolism of shellfish sterols in a patient with sitosterolemia and xanthomatosis. J. Clin. Invest. 77: 1864-1872.
18. Schoenheimer, R. 1931. New contributions in sterol metabolism. Science. 74: 579-584.
19. Gould, R. G., R. J. Jones, G. V. LeRoy, R. W. Wissler, and C. B. Taylor. 1969. Absorbability of beta-skosterol in humans. Metabolism. 18: 652-662.
20. Salen, G., E. H. Ahrens, Jr., and S. M. Grundy. 1970. Metabolism of beta-sitosterol in man. J. Clin. Invest. 49: 952-967.
21. Yu, L., K. Von Bergmann, D. Lutjohann, H. H. Hobbs, and J. C. Cohen. 2004. Selective sterol accumulation in ABCG5/ABCG8-deficient mice. J. Lipid Res. 45: 301-307.
22. Sudhop, T., Y. Sahin, B. Lindenthal, C. Hahn, C. Luers, H. K. Berthold, and K. von Bergmann. 2002. Comparison of the hepatic clearances of campesterol, sitosterol, and cholesterol in healthy subjects suggests that efflux transporters controlling intestinal sterol absorption also regulate biliary secretion. Gut. 51: 860-863.
23. Yang, C., L. Yu, W. Li, F. Xu, J. C. Cohen, and H. H. Hobbs. 2004. Disruption of cholesterol homeostasis by plant sterols. J. Clin. Invest. 114: 813-822.
24. Dayal, B., N. H. Ertel, K. R. Rapole, A. Asgaonkar, and G. Salen. 1997. Rapid hydrogenation of unsaturated sterols and bile alcohols using microwaves. Steroids. 62: 451-454.
25. Yu, L., R. E. Hammer, J. Li-Hawkins, K. Von Bergmann, D. Lutjohann, J. C. Cohen, and H. H. Hobbs. 2002. Disruption of Abcg5 and Abcg8 in mice reveals their crucial role in biliary cholesterol secretion. Proc. Natl. Acad. Sci. USA. 99: 16237-16242.
26. Yu, L., J. Li-Hawkins, R. E. Hammer, K. E. Berge, J. D. Horton, J. C. Cohen, and H. H. Hobbs. 2002. Overexpression of ABCG5 and ABCG8 promotes biliary cholesterol secretion and reduces fractional absorption of dietary cholesterol. J. Clin. Invest. 110: 671-680.
27. Lutjohann, D., O. Breuer, G. Ahlborg, I. Nennesmo, A. Siden, U. Diczfalusy, and I. Bjorkhem. 1996. Cholesterol homeostasis in human brain: evidence for an age-dependent flux of 24S-hydroxycholesterol from the brain into the circulation. Proc. Natl. Acad. Sci. USA. 93: 9799-9804.
28. Lutjohann, D., A. Brzezinka, E. Barth, D. Abramowski, M. Staufenbiel, K. von Bergmann, K. Beyreuther, G. Multhaup, and T. A. Bayer. 2002. Profile of cholesterol-related sterols in aged amyloid precursor protein transgenic mouse brain. J. Lipid Res. 43: 1078-1085.
29. Lutjohann, D., I. Bjorkhem, and L. Ose. 1996. Phytosterolaemia in a Norwegian family: diagnosis and characterization of the first Scandinavian case. Scand. J. Clin. Lab. Invest. 56: 229-240.
30. Repa, J. J., J. M. Dietschy, and S. D. Turley. 2002. Inhibition of cholesterol absorption by SCH 58053 in the mouse is not mediated via changes in the expression of mRNA for ABCA1, ABCG5, or ABCG8 in the enterocyte. J. Lipid Res. 43: 1864-1874.
31. Honda, A., G. Salen, L. B. Nguyen, G. S. Tint, A. K. Batta, and S. Shefer. 1998. Down-regulation of cholesterol biosynthesis in sitosterolemia: diminished activities of acetoacetyl-CoA thiolase, 3-hydroxy-3-methylglutaryl- CoA synthase, reductase, squalene synthase, and 7-dehydrocholesterol delta7-reductase in liver and mononuclear leukocytes. J. Lipid Res. 39: 44-50.
32. Salen, G., K. von Bergmann, D. Lutjohann, P. Kwiterovich, J. Kane, S. B. Patel, T. Musliner, P. Stein, and B. Musser. 2004. Ezetimibe effectively reduces plasma plant sterols in patients with sitosterolemia. Circulation. 109: 966-971.
33. Wilund, K. R., L. Yu, F. Xu, G. Vega, S. Grundy, J. C. Cohen, and H. H. Hobbs. 2004. Plant sterol levels are not associated with atherosclerosis in mice and men. Arterioscler. Thromb. Vasc. Biol. 12: 2326-2332.