Ezetimibe markedly attenuates hepatic cholesterol accumulation and improves liver function in the lysosomal acid lipase-deficient mouse, a model for cholesteryl ester storage disease

Biochemical and Biophysical Research Communications

Volumn 443, Issue 3, 2014, Pages 1073-1077

  Chuang, Jen Chieh ^a   Lopez, Adam M ^a   Posey, Kenneth S ^a   Turley, Stephen D ^a  

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

Author keywords

Cholesterol absorption; Fatty liver; Hepatomegaly; Intrahepatic; Small intestine; Triacylglycerol

Indexed keywords

ACID LIPASE; ALANINE AMINOTRANSFERASE; CHOLESTEROL; CHOLESTEROL ESTER; EZETIMIBE; LYSOSOMAL ACID LIPASE; UNCLASSIFIED DRUG;

ADULT; ALANINE AMINOTRANSFERASE BLOOD LEVEL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; ARTIFICIAL DIET; CHOLESTEROL ESTER STORAGE DISEASE; CHOLESTEROL LIVER LEVEL; CONCENTRATION (PARAMETERS); CONTROLLED STUDY; ENZYME ACTIVITY; FEMALE; INTESTINE FUNCTION; LIPID STORAGE; LIVER FUNCTION; LIVER MASS; LIVER WEIGHT; MALE; MOUSE; NONHUMAN; PRIORITY JOURNAL; RAT; YOUNG ADULT;

MUS; RATTUS;

ALANINE AMINOTRANSFERASE; ALT; CE; CHOLESTEROL ABSORPTION; CHOLESTERYL ESTER; FATTY LIVER; GENE THAT ENCODES LAL; HEPATOMEGALY; INTRAHEPATIC; LAL; LIPA; LYSOSOMAL ACID LIPASE; NIEMANN-PICK C1-LIKE1; NPC1L1; SI; SMALL INTESTINE; TAG; TRIACYLGLYCEROL; UC; UNESTERIFIED CHOLESTEROL;

ANIMALS; AZETIDINES; BODY WEIGHT; CHOLESTEROL; CHOLESTEROL ESTER STORAGE DISEASE; DISEASE MODELS, ANIMAL; HEPATOMEGALY; INTESTINES; LIVER; LIVER FUNCTION TESTS; MALE; MICE; ORGAN SIZE; RATS; STEROL ESTERASE; TRIGLYCERIDES;

EID: 84893694377     PISSN: 0006291X     EISSN: 10902104     Source Type: Journal    
DOI: 10.1016/j.bbrc.2013.12.096     Document Type: Article

References (36)

1. M.S. Brown, and J.L. Goldstein Receptor-mediated endocytosis: insights from the lipoprotein receptor system Proc. Natl. Acad. Sci. U.S.A. 76 1979 3330 3337
2. B. Liu, C. Xie, J.A. Richardson, S.D. Turley, and J.M. Dietschy Receptor-mediated and bulk-phase endocytosis cause macrophage and cholesterol accumulation in Niemann-Pick C disease J. Lipid Res. 48 2007 1710 1723
3. J.L. Goldstein, S.E. Dana, J.R. Faust, A.L. Beaudet, and M.S. Brown Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein. Observations in cultured fibroblasts from a patient with cholesteryl ester storage disease J. Biol. Chem. 250 1975 8487 8495
4. G.A. Grabowski, and H. Du Lysosomal acid lipase deficiencies: the wolman disease/cholesteryl ester storage disease spectrum D. Valle, A. Beaudet, B. Vogelstein, K. Kinzler, S. Antonarakis, A. Ballabio, Scriver's Online Metabolic and Molecular Basis of Inherited Metabolic Disease 2012 McGraw-Hill
5. D.L. Bernstein, H. Hulkova, M.G. Bialer, and R.J. Desnick Cholesteryl ester storage disease: review of the findings in 135 reported patients with an underdiagnosed disease J. Hepatol. 58 2013 1230 1243
6. M. Kuriyama, H. Yoshida, M. Suzuki, J. Fujiyama, and A. Igata Lysosomal acid lipase deficiency in rats: lipid analyses and lipase activities in liver and spleen J. Lipid Res. 31 1990 1605 1612
7. H. Du, M. Duanmu, D. Witte, and G.A. Grabowski Targeted disruption of the mouse lysosomal acid lipase gene: long-term survival with massive cholesteryl ester and triglyceride storage Hum. Mol. Genet. 7 1998 1347 1354
8. H. Du, M. Heur, M. Duanmu, G.A. Grabowski, D.Y. Hui, D.P. Witte, and J. Mishra Lysosomal acid lipase-deficient mice: depletion of white and brown fat, severe hepatosplenomegaly, and shortened life span J. Lipid Res. 42 2001 489 500
9. X. Lian, C. Yan, L. Yang, Y. Xu, and H. Du Lysosomal acid lipase deficiency causes respiratory inflammation and destruction in the lung Am. J. Physiol. Lung Cell. Mol. Physiol. 286 2004 L801 807
10. J.M. Dietschy, S.D. Turley, and D.K. Spady Role of liver in the maintenance of cholesterol and low density lipoprotein homeostasis in different animal species, including humans J. Lipid Res. 34 1993 1637 1659
11. E.M. Foley, P.L. Gordts, K.I. Stanford, J.C. Gonzales, R. Lawrence, N. Stoddard, and J.D. Esko Hepatic remnant lipoprotein clearance by heparan sulfate proteoglycans and low-density lipoprotein receptors depend on dietary conditions in mice Arterioscler. Thromb. Vasc. Biol. 33 2013 2065 2074
12. A.D. Cooper Hepatic uptake of chylomicron remnants J. Lipid Res. 38 1997 2173 2192
13. T.M. Nguyen, J.K. Sawyer, K.L. Kelley, M.A. Davis, and L.L. Rudel Cholesterol esterification by ACAT2 is essential for efficient intestinal cholesterol absorption: evidence from thoracic lymph duct cannulation J. Lipid Res. 53 2012 95 104
14. M.A. Valasek, J.J. Repa, G. Quan, J.M. Dietschy, and S.D. Turley Inhibiting intestinal NPC1L1 activity prevents diet-induced increase in biliary cholesterol in Golden Syrian hamsters Am. J. Physiol. Gastrointest. Liver Physiol. 295 2008 G813 822
15. S.D. Turley, M.A. Valasek, J.J. Repa, and J.M. Dietschy Multiple mechanisms limit the accumulation of unesterified cholesterol in the small intestine of mice deficient in both ACAT2 and ABCA1 Am. J. Physiol. Gastrointest. Liver Physiol. 299 2010 G1012 1022
16. L.J. Engelking, M.R. McFarlane, C.K. Li, and G. Liang Blockade of cholesterol absorption by ezetimibe reveals a complex homeostatic network in enterocytes J. Lipid Res. 53 2012 1359 1368
17. D.C. Chan, G.F. Watts, S.K. Gan, E.M. Ooi, and P.H. Barrett Effect of ezetimibe on hepatic fat, inflammatory markers, and apolipoprotein B-100 kinetics in insulin-resistant obese subjects on a weight loss diet Diabetes Care 33 2010 1134 1139
18. O. de Bari, B.A. Neuschwander-Tetri, M. Liu, P. Portincasa, and D.Q. Wang Ezetimibe: its novel effects on the prevention and the treatment of cholesterol gallstones and nonalcoholic fatty liver disease J. Lipids 2012 2012 302847
19. M. Deushi, M. Nomura, A. Kawakami, M. Haraguchi, M. Ito, M. Okazaki, H. Ishii, and M. Yoshida Ezetimibe improves liver steatosis and insulin resistance in obese rat model of metabolic syndrome FEBS Lett. 581 2007 5664 5670
20. E.D. Labonte, L.M. Camarota, J.C. Rojas, R.J. Jandacek, D.E. Gilham, J.P. Davies, Y.A. Ioannou, P. Tso, D.Y. Hui, and P.N. Howles Reduced absorption of saturated fatty acids and resistance to diet-induced obesity and diabetes by ezetimibe-treated and Npc1l1-/- mice Am. J. Physiol. Gastrointest. Liver Physiol. 295 2008 G776 783
21. S. Zheng, L. Hoos, J. Cook, G. Tetzloff, H. Davis Jr., M. van Heek, and J.J. Hwa Ezetimibe improves high fat and cholesterol diet-induced non-alcoholic fatty liver disease in mice Eur. J. Pharmacol. 584 2008 118 124
22. L. Jia, Y. Ma, S. Rong, J.L. Betters, P. Xie, S. Chung, N. Wang, W. Tang, and L. Yu Niemann-Pick C1-Like 1 deletion in mice prevents high-fat diet-induced fatty liver by reducing lipogenesis J. Lipid Res. 51 2010 3135 3144
23. T. Matono, M. Koda, S. Tokunaga, J. Kato, T. Sugihara, M. Ueki, and Y. Murawaki Therapeutic effects of ezetimibe for non-alcoholic steatohepatitis in Fatty Liver Shionogi-ob/ob mice Hepatol. Res. 41 2011 1240 1248
24. M. Naples, C. Baker, M. Lino, J. Iqbal, M.M. Hussain, and K. Adeli Ezetimibe ameliorates intestinal chylomicron overproduction and improves glucose tolerance in a diet-induced hamster model of insulin resistance Am. J. Physiol. Gastrointest. Liver Physiol. 302 2012 G1043 1052
25. M. Ushio, Y. Nishio, O. Sekine, Y. Nagai, Y. Maeno, S. Ugi, T. Yoshizaki, K. Morino, S. Kume, A. Kashiwagi, and H. Maegawa Ezetimibe prevents hepatic steatosis induced by a high-fat but not a high-fructose diet Am. J. Physiol. Endocrinol. Metab. 305 2013 E293 304
26. X. Wang, K. Sugimoto, T. Fujisawa, N. Shindo, S. Minato, Y. Kamada, M. Hamano, M. Ohishi, H. Ikegami, and H. Rakugi Novel effect of ezetimibe to inhibit the development of non-alcoholic fatty liver disease in Fatty Liver Shionogi mouse Hepatol. Res. 2013 10.1111/hepr.12092 in press
27. E.P. Beltroy, B. Liu, J.M. Dietschy, and S.D. Turley Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease J. Lipid Res. 48 2007 869 881
28. C.M. Ramirez, B. Liu, A. Aqul, A.M. Taylor, J.J. Repa, S.D. Turley, and J.M. Dietschy Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations J. Lipid Res. 52 2011 688 698
29. J.R. Crouse, S.M. Grundy, and J.H. Johnson Effects of AOMA on cholesterol metabolism in man Metabolism 31 1982 733 739
30. J.J. Repa, S.D. Turley, G. Quan, and J.M. Dietschy Delineation of molecular changes in intrahepatic cholesterol metabolism resulting from diminished cholesterol absorption J. Lipid Res. 46 2005 779 789
31. M. Schwarz, D.W. Russell, J.M. Dietschy, and S.D. Turley Marked reduction in bile acid synthesis in cholesterol 7α-hydroxylase-deficient mice does not lead to diminished tissue cholesterol turnover or to hypercholesterolemia J. Lipid Res. 39 1998 1833 1843
32. S.D. Turley, M.W. Herndon, and J.M. Dietschy Reevaluation and application of the dual-isotope plasma ratio method for the measurement of intestinal cholesterol absorption in the hamster J. Lipid Res. 35 1994 328 339
33. B. Liu Therapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease Clin. Lipidol. 7 2012 289 301
34. H.N. Ginsberg, N.A. Le, M.P. Short, R. Ramakrishnan, and R.J. Desnick Suppression of apolipoprotein B production during treatment of cholesteryl ester storage disease with lovastatin. Implications for regulation of apolipoprotein B synthesis J. Clin. Invest. 80 1987 1692 1697
35. T. Sudhop, M. Reber, D. Tribble, A. Sapre, W. Taggart, P. Gibbons, T. Musliner, K. von Bergmann, and D. Lutjohann Changes in cholesterol absorption and cholesterol synthesis caused by ezetimibe and/or simvastatin in men J. Lipid Res. 50 2009 2117 2123
36. V.T. Tadiboyina, D.M. Liu, B.A. Miskie, J. Wang, and R.A. Hegele Treatment of dyslipidemia with lovastatin and ezetimibe in an adolescent with cholesterol ester storage disease Lipids Health Dis. 4 2005 1 6