Abnormal hepatobiliary and circulating lipid metabolism in the Long-Evans Cinnamon rat model of Wilson's disease

Life Sciences

Volumn 80, Issue 16, 2007, Pages 1472-1483

  Levy, Emile ^a   Brunet, Sylvain ^a   Alvarez, Fernando ^a   Seidman, Ernest ^a   Bouchard, Guylaine ^a   Escobar, Enrique ^a   Martin, Steve ^a  

^a UNIVERSITY OF MONTREAL   (Canada)

Author keywords

Apo B; Lipid peroxidation; Liver copper; MTP; Sterol metabolism

Indexed keywords

APOLIPOPROTEIN; BILE ACID; CHOLESTEROL; CHOLESTEROL 7ALPHA MONOOXYGENASE; CHOLESTEROL ACYLTRANSFERASE; CHOLESTEROL ESTER; HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE; LIPOPROTEIN; MALONALDEHYDE; TRIACYLGLYCEROL;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BILIARY TRACT DRAINAGE; CONTROLLED STUDY; ENZYME ACTIVITY; GENETIC DISORDER; HOMEOSTASIS; HYPOCHOLESTEROLEMIA; LIPID BLOOD LEVEL; LIPID METABOLISM; LIPID PEROXIDATION; LIPOPROTEIN METABOLISM; LIVER; NONHUMAN; PROTEIN EXPRESSION; RAT; STEATOSIS; STEROL METABOLISM; WILSON DISEASE;

CINNAMOMUM VERUM; RATTUS;

EID: 33947135813     PISSN: 00243205     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.lfs.2007.01.017     Document Type: Article

References (65)

1. Bacon B.R., and Schilsky M.L. New knowledge of genetic pathogenesis of hemochromatosis and Wilson's disease. Advances in Internal Medicine 44 (1999) 91-116
2. Bartlett G.R. Phosphorus assay in column chromatography. Journal of Biological Chemistry 234 3 (1959) 466-468
3. Bielicki J.K., and Forte T.M. Evidence that lipid hydroperoxides inhibit plasma lecithin : cholesterol acyltransferase activity. Journal of Lipid Research 40 5 (1999) 948-954
4. Brown M.S., Goldstein J.L., and Dietschy J.M. Active and inactive forms of 3-hydroxy-3-methylglutaryl coenzyme A reductase in the liver of the rat. Comparison with the rate of cholesterol synthesis in different physiological states. Journal of Biological Chemistry 254 12 (1979) 5144-5149
5. Brunet S., Thibault L., Delvin E., Yotov W., Bendayan M., and Levy E. Dietary iron overload and induced lipid peroxidation are associated with impaired plasma lipid transport and hepatic sterol metabolism in rats. Hepatology 29 6 (1999) 1809-1817
6. Brunet S., Thibault L., Lepage G., Seidman E.G., Dube N., and Levy E. Modulation of endoplasmic reticulum-bound cholesterol regulatory enzymes by iron/ascorbate-mediated lipid peroxidation. Free Radical Biology & Medicine 28 1 (2000) 46-54
7. Bull P.C., Thomas G.R., Rommens J.M., Forbes J.R., and Cox D.W. The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nature Genetics 5 4 (1993) 327-337
8. Burnett J.R., Wilcox L.J., and Huff M.W. Acyl coenzyme A: cholesterol acyltransferase inhibition and hepatic apolipoprotein B secretion. Clinica Chimica Acta 286 1-2 (1999) 231-242
9. Cooper A.D. Role of the liver in the degradation of lipoproteins. Gastroenterology 88 1 Pt 1 (1985) 192-205
10. Culotta V.C., and Gitlin J.D. Disorders of copper transport. In: Scriver C.R., Beaudet A.L., Sly S.W., and Valley D. (Eds). The Molecular and Metabolic Basis of Inherited Disease (2001), McGraw-Hill, New York 3105-3126
11. Danks D.M. Disorders of copper transport. In: Scriver C.R., Beaudet A.L., Sly S.W., and Valley D. (Eds). The Metabolic and Molecular Basis of Inherited Disease (1995), McGraw-Hill, New York 2211-2235
12. Graf G.A., Yu L., Li W.P., Gerard R., Tuma P.L., Cohen J.C., and Hobbs H.H. ABCG5 and ABCG8 are obligate heterodimers for protein trafficking and biliary cholesterol excretion. Journal of Biological Chemistry 278 48 (2003) 48275-48282
13. Halliwell B., and Gutteridge J.M.C. Lipid peroxidation: a radical chain reaction. In: Halliwell B. (Ed). Free Radicals in Biology and Medicine (1985), Clarendon Press, Oxford 139-189
14. Harada M., Sakisaka S., Terada K., Kimura R., Kawaguchi T., Koga H., Taniguchi E., Sasatomi K., Miura N., Suganuma T., Fujita H., Furuta K., Tanikawa K., Sugiyama T., and Sata M. Role of ATP7B in biliary copper excretion in a human hepatoma cell line and normal rat hepatocytes. Gastroenterology 118 5 (2000) 921-928
15. Huff M.W., and Burnett J.R. 3-Hydroxy-3-methylglutaryl coenzyme A reductase inhibitors and hepatic apolipoprotein B secretion. Current Opinion in Lipidology 8 3 (1997) 138-145
16. Hung I.H., Suzuki M., Yamaguchi Y., Yuan D.S., Klausner R.D., and Gitlin J.D. Biochemical characterization of the Wilson disease protein and functional expression in the yeast Saccharomyces cerevisiae. Journal of Biological Chemistry 272 34 (1997) 21461-21466
17. Hussain S.P., Raja K., Amstad P.A., Sawyer M., Trudel L.J., Wogan G.N., Hofseth L.J., Shields P.G., Billiar T.R., Trautwein C., Hohler T., Galle P.R., Phillips D.H., Markin R., Marrogi A.J., and Harris C.C. Increased p53 mutation load in nontumorous human liver of Wilson disease and hemochromatosis: oxyradical overload diseases. Proceedings of the National Academy of Sciences of the United States of America 97 23 (2000) 12770-12775
18. 14C]cholesterol as substrate. Analytical Biochemistry 182 2 (1989) 212-216
19. Jenkins K.J., and Kramer J.K. Influence of excess dietary copper on lipid composition of calf tissues. Journal of Dairy Science 72 10 (1989) 2582-2591
20. Jonas A. Lecithin cholesterol acyltransferase. Biochimica et Biophysica Acta 1529 1-3 (2000) 245-256
21. Kang S., and Davis R.A. Cholesterol and hepatic lipoprotein assembly and secretion. Biochimica et Biophysica Acta 1529 1-3 (2000) 223-230
22. Kendrick J.S., Wilkinson J., Cartwright I.J., Lawrence S., and Higgins J.A. Regulation of the assembly and secretion of very low density lipoproteins by the liver. Biological Chemistry 379 8-9 (1998) 1033-1040
23. 2 mixed-function oxidation system. Journal of Biological Chemistry 263 10 (1988) 4704-4711
24. Klett E.L., Lee M.H., Adams D.B., Chavin K.D., and Patel S.B. Localization of ABCG5 and ABCG8 proteins in human liver, gall bladder and intestine. BMC. Gastroenterology 4 1 (2004) 21
25. Kullak-Ublick G.A., Stieger B., and Meier P.J. Enterohepatic bile salt transporters in normal physiology and liver disease. Gastroenterology 126 1 (2004) 322-342
26. Lepage G., Munoz G., Champagne J., and Roy C.C. Preparative steps necessary for the accurate measurement of malondialdehyde by high-performance liquid chromatography. Analytical Biochemistry 197 2 (1991) 277-283
27. Levy E., Lepage G., Bendayan M., Ronco N., Thibault L., Galeano N., Smith L., and Roy C.C. Relationship of decreased hepatic lipase activity and lipoprotein abnormalities to essential fatty acid deficiency in cystic fibrosis patients. Journal of Lipid Research 30 8 (1989) 1197-1209
28. Levy E., Thibault L., Garofalo C., Messier M., Lepage G., Ronco N., and Roy C.C. Combined (n-3 and n-6) essential fatty deficiency is a potent modulator of plasma lipids, lipoprotein composition, and lipolytic enzymes. Journal of Lipid Research 31 11 (1990) 2009-2017
29. Levy E., Garofalo C., Thibault L., Dionne S., Daoust L., Lepage G., and Roy C.C. Intraluminal and intracellular phases of fat absorption are impaired in essential fatty acid deficiency. American Journal of Physiology 262 2 Pt 1 (1992) G319-G326
30. Levy E., Bendayan M., Thibault L., Lambert M., and Paradis K. Lipoprotein abnormalities in two children with minimal biliary excretion. Journal of Pediatric Gastroenterology and Nutrition 20 4 (1995) 432-439
31. Levy E., Garofalo C., Rouleau T., Gavino V., and Bendayan M. Impact of essential fatty acid deficiency on hepatic sterol metabolism in rats. Hepatology 23 4 (1996) 848-857
32. Levy E., Menard D., Delvin E., Stan S., Mitchell G., Lambert M., Ziv E., Feoli-Fonseca J.C., and Seidman E. The polymorphism at codon 54 of the FABP2 gene increases fat absorption in human intestinal explants. Journal of Biological Chemistry 276 43 (2001) 39679-39684
33. Levy E., Stan S., Garofalo C., Delvin E.E., Seidman E.G., Menard D., 2001a. Immunolocalization, ontogeny, and regulation of microsomal triglyceride transfer protein in human fetal intestine. American Journal of Physiology. Gastrointestinal and Liver Physiology 280 (4), G563-G571.
34. Levy E., Stan S., Delvin E., Menard D., Shoulders C., Garofalo C., Slight I., Seidman E., Mayer G., and Bendayan M. Localization of microsomal triglyceride transfer protein in the Golgi: possible role in the assembly of chylomicrons. Journal of Biological Chemistry 277 19 (2002) 16470-16477
35. Lowry O.H., Rosebrough N.J., Farr A.L., and Randall R.J. Protein measurement with the Folin phenol reagent. Journal of Biological Chemistry 193 1 (1951) 265-275
36. Ma Y., Zhang D., Kawabata T., Kiriu T., Toyokuni S., Uchida K., and Okada S. Copper and iron-induced oxidative damage in non-tumor bearing LEC rats. Pathology International 47 4 (1997) 203-208
37. Mason T.M. The role of factors that regulate the synthesis and secretion of very-low-density lipoprotein by hepatocytes. Critical Reviews in Clinical Laboratory Sciences 35 6 (1998) 461-487
38. Maziere J.C., Myara I., Salmon S., Auclair M., Haigle J., Santus R., and Maziere C. Copper- and malondialdehyde-induced modification of high density lipoprotein and parallel loss of lecithin cholesterol acyltransferase activation. Atherosclerosis 104 1-2 (1993) 213-219
39. McCall M.R., Tang J.Y., Bielicki J.K., and Forte T.M. Inhibition of lecithin-cholesterol acyltransferase and modification of HDL apolipoproteins by aldehydes. Arteriosclerosis, Thrombosis, and Vascular Biology 15 10 (1995) 1599-1606
40. McCall M.R., Carr A.C., Forte T.M., and Frei B. LDL modified by hypochlorous acid is a potent inhibitor of lecithin-cholesterol acyltransferase activity. Arteriosclerosis, Thrombosis, and Vascular Biology 21 6 (2001) 1040-1045
41. Mitchell D.M., Zhou M., Pariyarath R., Wang H., Aitchison J.D., Ginsberg H.N., and Fisher E.A. Apoprotein B100 has a prolonged interaction with the translocon during which its lipidation and translocation change from dependence on the microsomal triglyceride transfer protein to independence. Proceedings of the National Academy of Sciences of the United States of America. 95 25 (1998) 14733-14738
42. Mori M., Hattori A., Sawaki M., Tsuzuki N., Sawada N., Oyamada M., Sugawara N., and Enomoto K. The LEC rat: a model for human hepatitis, liver cancer, and much more. American Journal of Pathology 144 1 (1994) 200-204
43. Myers B.M., Prendergast F.G., Holman R., Kuntz S.M., and LaRusso N.F. Alterations in the structure, physicochemical properties, and pH of hepatocyte lysosomes in experimental iron overload. Journal of Clinical Investigation 88 4 (1991) 1207-1215
44. Noe J., Stieger B., and Meier P.J. Functional expression of the canalicular bile salt export pump of human liver. Gastroenterology 123 5 (2002) 1659-1666
45. Okayasu T., Tochimaru H., Hyuga T., Takahashi T., Takekoshi Y., Li Y., Togashi Y., Takeichi N., Kasai N., and Arashima S. Inherited copper toxicity in Long-Evans cinnamon rats exhibiting spontaneous hepatitis: a model of Wilson's disease. Pediatric Research 31 3 (1992) 253-257
46. Packard C.J., and Shepherd J. The hepatobiliary axis and lipoprotein metabolism: effects of bile acid sequestrants and ileal bypass surgery. Journal of Lipid Research 23 8 (1982) 1081-1098
47. Pena M.M., Lee J., and Thiele D.J. A delicate balance: homeostatic control of copper uptake and distribution. Journal of Nutrition 129 7 (1999) 1251-1260
48. Raabe M., Flynn L.M., Zlot C.H., Wong J.S., Veniant M.M., Hamilton R.L., and Young S.G. Knockout of the abetalipoproteinemia gene in mice: reduced lipoprotein secretion in heterozygotes and embryonic lethality in homozygotes. Proceedings of the National Academy of Sciences of the United States of America 95 15 (1998) 8686-8691
49. Roberts E.A., and Schilsky M.L. A practice guideline on Wilson disease. Hepatology 37 6 (2003) 1475-1492
50. Rodo M., Czonkowska A., Pulawska M., Swiderska M., Tarnacka B., and Wehr H. The level of serum lipids, vitamin E and low density lipoprotein oxidation in Wilson's disease patients. European Journal of Neurology 7 5 (2000) 491-494
51. Roelofsen H., Wolters H., Van Luyn M.J., Miura N., Kuipers F., and Vonk R.J. Copper-induced apical trafficking of ATP7B in polarized hepatoma cells provides a mechanism for biliary copper excretion. Gastroenterology 119 3 (2000) 782-793
52. Samuni A., Chevion M., and Czapski G. Unusual copper-induced sensitization of the biological damage due to superoxide radicals. Journal of Biological Chemistry 256 24 (1981) 12632-12635
53. Schaefer M., Hopkins R.G., Failla M.L., and Gitlin J.D. Hepatocyte-specific localization and copper-dependent trafficking of the Wilson's disease protein in the liver. American Journal of Physiology 276 3 Pt 1 (1999) G639-G646
54. Schosinsky K.H., Lehmann H.P., and Beeler M.F. Measurement of ceruloplasmin from its oxidase activity in serum by use of o-dianisidine dihydrochloride. Clinical Chemistry 20 12 (1974) 1556-1563
55. Seidel D. Lipoproteins in liver disease. Journal of Clinical Chemistry and Clinical Biochemistry 25 9 (1987) 541-551
56. Sternlieb I. Wilson's disease. Clinics in Liver Disease 4 1 (2000) 229-2ix
57. Taborsky G. Oxidative modification of proteins in the presence of ferrous ion and air. Effect of ionic constituents of the reaction medium on the nature of the oxidation products. Biochemistry 12 7 (1973) 1341-1348
58. Taniguchi M., Sugiyama T., and Taniguchi N. Abnormal lipid metabolism in LEC rats. In: Mori M., Yoshida M.C., Takeichi N., and Taniguchi N. (Eds). The LEC Rat, A New Model for Hepatitis and Liver Cancer (1991), Springer-Verlag, Tokyo 169-174
59. Tietze F. Enzymic method for quantitative determination of nanogram amounts of total and oxidized glutathione: applications to mammalian blood and other tissues. Analytical Biochemistry 27 3 (1969) 502-522
60. Turley S.D., and Dietschy J.M. The metabolism and excretion of cholesterol by the liver. In: Arias I.M., Jacoby H., Popper H., Schachter D., and Schafritz D.A. (Eds). The Liver: Biology and Pathology (1988), Raven, New York 617-641
61. Wang S.L., Du E.Z., Martin T.D., and Davis R.A. Coordinate regulation of lipogenesis, the assembly and secretion of apolipoprotein B-containing lipoproteins by sterol response element binding protein 1. Journal of Biological Chemistry 272 31 (1997) 19351-19358
62. Wanon J., Guertin F., Brunet S., Delvin E., Gavino V., Bouthillier D., Lairon D., Yotov W., and Levy E. The effects of cholesterol uptake from high-density lipoprotein subfractions on biliary sterol secretion in rats with essential fatty-acid deficiency. Hepatology 27 3 (1998) 779-786
63. Wetterau J.R., Aggerbeck L.P., Bouma M.E., Eisenberg C., Munck A., Hermier M., Schmitz J., Gay G., Rader D.J., and Gregg R.E. Absence of microsomal triglyceride transfer protein in individuals with abetalipoproteinemia. Science 258 5084 (1992) 999-1001
64. Wu J., Forbes J.R., Chen H.S., and Cox D.W. The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene. Nature Genetics 7 4 (1994) 541-545
65. Yu L., Gupta S., Xu F., Liverman A.D., Moschetta A., Mangelsdorf D.J., Repa J.J., Hobbs H.H., and Cohen J.C. Expression of ABCG5 and ABCG8 is required for regulation of biliary cholesterol secretion. Journal of Biological Chemistry 280 10 (2005) 8742-8747