Liver disease with altered bile acid transport in Niemann-Pick C mice on a high-fat, 1% cholesterol diet

American Journal of Physiology - Gastrointestinal and Liver Physiology

Volumn 289, Issue 2 52-2, 2005, Pages

  Erickson, Robert P ^a,b   Bhattacharyya, Achyut ^a   Hunter, Robert J ^a   Heidenreich, Randall A ^a   Cherrington, Nathan J ^a  

^a UNIVERSITY OF ARIZONA   (United States)

^b UNIVERSITY OF ARIZONA COLLEGE OF MEDICINE   (United States)

Author keywords

Bile acid transporters; Low density lipoprotein receptor knockout; Neonatal cholestasis; Niemann Pick C; Non zone dependent fibrosis

Indexed keywords

ALANINE AMINOTRANSFERASE; BSEP PROTEIN; CARRIER PROTEIN; CHOLESTEROL; GAMMA GLUTAMYLTRANSFERASE; LIPID; LOW DENSITY LIPOPROTEIN RECEPTOR; MULTIDRUG RESISTANCE PROTEIN 1; MULTIDRUG RESISTANCE PROTEIN 2; MULTIDRUG RESISTANCE PROTEIN 3; MULTIDRUG RESISTANCE PROTEIN 4; MULTIDRUG RESISTANCE PROTEIN 5; NTCP PROTEIN; OATP1 PROTEIN; OATP2 PROTEIN; OATP4 PROTEIN; UNCLASSIFIED DRUG;

ALANINE AMINOTRANSFERASE BLOOD LEVEL; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BILE ACID METABOLISM; BILE ACID TRANSPORT; CHOLESTATIC HEPATITIS; CHOLESTEROL BLOOD LEVEL; CHOLESTEROL DIET; CONTROLLED STUDY; DEATH; FEMALE; HEPATOMEGALY; HISTOPATHOLOGY; KNOCKOUT GENE; LIPID DIET; LIPID LIVER LEVEL; LIVER CELL; LIVER DISEASE; MACROPHAGE; MALE; NEONATAL CHOLESTASIS; NEWBORN DISEASE; NIEMANN PICK DISEASE; NONHUMAN; NPC1 GENE; PRIORITY JOURNAL; PROTEIN CONTENT; REAL TIME POLYMERASE CHAIN REACTION;

ANIMALS; BILE ACIDS AND SALTS; CHOLESTEROL, DIETARY; FEMALE; HEPATITIS; MALE; MICE; MICE, INBRED BALB C; MICE, KNOCKOUT; NIEMANN-PICK DISEASES; ORGANIC ANION TRANSPORTERS, SODIUM-INDEPENDENT; ORGANIC CATION TRANSPORT PROTEINS; PROTEINS; RECEPTORS, LDL;

EID: 22544485069     PISSN: 01931857     EISSN: None     Source Type: Journal    
DOI: 10.1152/ajpgi.00568.2004     Document Type: Article

References (33)

1. Aleksunes LM, Slitt AL, Cherrington NJ, Thibodeau MS, Klaassen CD, and Manautou JE. Differential expression of mouse hepatic transporter genes in response to acetaminophen and carbon tetrachloride. Tox Sci 83: 44-52, 2005.
2. Amigo L, Mendoza H, Castro J, Quinones V, Miquel JF, and Zanlungo S. Relevance of Niemann-Pick type C1 protein expression in controlling plasma cholesterol and biliary lipid secretion in mice. Hepatology 36: 819-828, 2002.
3. Bauer P, Knoblich R, Bauer C, Finckh U, Hufen A, Kropp J, Braun S, Kustermann-Kuhn B, Schmidt D, Harzer K, and Rolfs A. NPC1: Complete genomic sequence, mutation analysis, and characterization of haplotypes. Hum Mutat 19: 30-38, 2002.
4. Boison D, Scheurer L, Zumsteg V, Rulicke T, Litynski P, Fowler B, Brandner S, and Mohler H. Neonatal hepatic steatosis by disruption of the adenosine kinase gene. Proc Natl Acad Sci USA 99: 6985-6990, 2002.
5. Boles RG, Buck EA, Blitzer MG, Platt MS, Cowan TM, Martin SK, Yoon H, Madsen JA, Reyes-Mugica M, and Rinaldo P. Retrospective biochemical screening of fatty acid oxidation disorders in postmortem livers of 418 cases of sudden death in the first year of life. J Pediatr 132: 924-933, 1998.
6. Brivet M, Boutron A, Slama A, Costa C, Thuillier L, Demaugre F, Rabier D, Saudubray JM, and Bonnefont JP. Defects in activation and transport of fatty acids. J Inherit Metab Dis 22: 428-441, 1999.
7. Cherrington NJ, Slitt AL, Maher JM, Zhang XX, Zhang J, Huang W, Wan YJ, Moore DD, and Klaassen CD. Induction of multidrug resistance protein 3 (mrp3) in vivo is independent of constitutive androstane receptor. Drug Metab Dispos 31: 1315-1319, 2003.
8. Erickson RP, Carver WS, Camargo F, Hossain GS, and Heidenreich RA. Pharmacological and genetic modifications of somatic cholesterol do not substantially alter the course of CNS disease in Niemann-Pick C mice. J Inherit Metab Dis 23: 54-62, 2000.
9. Frolov A, Zielinski SE, Crowley JR, Dudley-Rucker N, Schaffer JE, and Ory DS. NPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterols. J Biol Chem 278: 25517-25525, 2003.
10. Fromenty B and Pessayre D. Impaired mitochondrial function in microvesicular steatosis. Effects of drugs, ethanol, hormones and cytokines. J Hepatol 26: 43-53, 1997.
11. Gaw A, Mancini FP, and Ishibashi S. Rapid genotyping of low density lipoprotein receptor knockout mice using a polymerase chain reaction technique. Lab Anim Sci 29: 447-449, 1995.
12. Guo J, Trouard TP, Galons JP, Erickson R, and Gillies RJ. Magnetization transfer contrast image in Niemann pick type C mouse liver. J Magn Reson Imaging 18: 321-327, 2003.
13. Heider JG and Boyett RL. The picomole determination of free and total cholesterol in cells in culture. J Lipid Res 19: 514-518, 1978.
14. Ishibashi S, Brown MS, Goldstein JL, Gerard RD, Hammer RE, and Herz J. Hypercholesterolemia in low density lipoprotein receptor knockout mice and its reversal by adenovirus-mediated gene delivery. J Clin Invest 92: 883-893, 1993.
15. Kelly DA, Portmann B, Mowat AP, Sherlock S, and Lake BD. Niemann-Pick disease type C: diagnosis and outcome in children, with particular reference to liver disease. J Pediatr 123: 242-247, 1993.
16. Loftus SK, Morris JA, Carstea ED, Gu JZ, Cummings C, Brown A, Ellison J, Ohno K, Rosenfeld MA, Tagle DA, Pentchev PG, and Pavan WJ. Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene. Science 277: 232-235, 1997.
17. Mieli-Vergani G, Howard ER, and Mowat AP. Liver disease in infancy: a 20-year perspective. Gut Suppl: S123-S128, 1991.
18. Miyawaki S, Mitsuoka S, Sakiyama T, and Kitagawa T. Sphingomyelinosis, a new mutation in the mouse: a model of Niemann-Pick disease in humans. J Hered 73: 257-263, 1982.
19. Miyawaki S, Mitsuoka S, Sakiyama T, and Kitagawa T. Time course of hepatic lipids accumulation in a strain of mice with an inherited deficiency of sphingomyelinase. J Hered 74: 465-468, 1983.
20. Morris MD, Bhuvaneswaran C, Shio H, and Fowler S. Lysosome lipid storage disorder in NCTR-BALB/c mice. I. Description of the disease and genetics. Am J Pathol 108: 140-149, 1982.
21. Parks DJ, Blanchard SG, Bledsoe RK, Chandra G, Consler TG, Kliewer SA, Stimmel JB, Willson TM, Zavacki AM, Moore DD, and Lehmann JM. Bile acids: natural ligands for an orphan nuclear receptor. Science 284: 1365-1368, 1999.
22. Pentchev PG, Boothe AD, Kruth HS, Weintroub H, Stivers J, and Brady RO. A genetic storage disorder in BALB/C mice with a metabolic block in esterification of exogenous cholesterol. J Biol Chem 259: 5784-5791, 1984.
23. Pentchev PG, Gal AE, Booth AD, Omodeo-Sale F, Fouks J, Neumeyer BA, Quirk JM, Dawson G, and Brady RO. A lysosomal storage disorder in mice characterized by a dual deficiency of sphingomyelinase and glucocerebrosidase. Biochim Biophys Acta 619: 669-679, 1980.
24. Pentchev PG, Boothe AD, Kruth HS, Weintroub H, Stivers J, and Brady RO. A genetic storage disorder in BALB/C mice with a metabolic block in esterification of exogenous cholesterol. J Biol Chem 259: 5784-5791, 1984.
25. Pentchev PG, Vanier MT, Suzuki K, and Patterson MC. Nieman-Pick disease type C. A cellular cholesterol lipidosis. In: The Metabolic and Molecular Basis of Inherited Disease (8th ed.), edited by Scriver CR, Beaudet AL, Sly WS, and Valle D. New York: McGraw-Hill, 1995, p. 5587-5627.
26. Repa JJ, Berge KE, Pomajzl C, Richardson JA, Hobbs H, and Mangelsdorf DJ. Regulation of ATP-binding cassette sterol transporters ABCG5 and ABCG8 by the liver X receptors alpha and beta. J Biol Chem 277: 18793-18800, 2002.
27. Shio H, Fowler S, Bhuvaneswaran C, and Morris MD. Lysosome lipid storage disorder in NCTR-BALB/c mice. II. Morphologic and cytochemical studies. Am J Pathol 108: 150-159, 1982.
28. Sinai CJ, Tohkin M, Miyata M, Ward JM, Lambert G, and Gonzalez FJ. Targeted disruption of the nuclear receptor FXR/BAR impairs bile acid and lipid homeostasis. Cell 102: 731-744, 2000.
29. van Nieuwerk CMJ, Groen AK, Ottenhoff R, van Wijland M, van den Bergh Weerman and MA, Thtgat GNJ, Offerhaus JJA, and Oude Elferink RPJ. The role of bile salt composition in liver pathology of mdr2 (-/-) mice: differences between males and females. J Hepatol 26: 138-145, 1997.
30. Wang R, Lam P, Liu L, Forrest D, Yousef IM, Mignault D, Phillips MJ, and Ling V. Severe Cholestasis induced by cholic acid feeding in knockout mice of sister of p-glycoprotein. Hepatology 38: 1489-1499, 2003.
31. Xie C, Turley SD, and Dietschy JM. Centripetal cholesterol flow from the extrahepatic organs through the liver is normal in mice with mutated Niemann-Pick type C protein (NPC1). J Lipid Res 41: 1278-1289, 2000.
32. Yerushalmi B, Sokol RJ, Narkewicz MR, Smith D, Ashmead JW, and Wenger DA. Niemann-Pick disease type C in neonatal cholestasis at a North American center. J Pediatr Gastroenterol Nutr 35: 44-50, 2002.
33. Yu L, Hammer RE, Li-Hawkins J, Von Bergmann K, Lutjohann D, Cohen JC, and Hobbs HH. Disruption of Abcg5 and Abcg8 in mice reveals their crucial role in biliary cholesterol secretion. Proc Natl Acad Sci USA 99: 16237-16242, 2002.