Relevance of Niemann-Pick type C1 protein expression in controlling plasma cholesterol and biliary lipid secretion in mice

Hepatology

Volumn 36, Issue 4 I, 2002, Pages 819-828

  Amigo, Ludwig ^a   Mendoza, Hegaly ^a   Castro, Juan ^a   Quiones, Verónica ^a   Miquel, Juan Francisco ^a   Zanlungo, Silvana ^a  

^a PONTIFICIA UNIVERSIDAD CATÓLICA DE CHILE   (Chile)

Author keywords

[No Author keywords available]

Indexed keywords

CHOLESTEROL; HIGH DENSITY LIPOPROTEIN; HIGH DENSITY LIPOPROTEIN CHOLESTEROL; LIPOPROTEIN; MEMBRANE PROTEIN; NIEMANN PICK TYPE C1 PROTEIN; UNCLASSIFIED DRUG;

ADENOVIRUS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BILE SECRETION; CELLULAR DISTRIBUTION; CHOLESTEROL BLOOD LEVEL; CHOLESTEROL DIET; CHOLESTEROL TRANSPORT; CONTROLLED STUDY; DISEASE MODEL; ENDOCYTOSIS; GENE TRANSFER; MOUSE; NIEMANN PICK DISEASE; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEFICIENCY; PROTEIN EXPRESSION;

ADENOVIRIDAE; ANIMAL FEED; ANIMALS; ATP-BINDING CASSETTE TRANSPORTERS; BILE DUCTS, EXTRAHEPATIC; CHOLESTEROL; CHOLESTEROL, DIETARY; GENE EXPRESSION; GENE TRANSFER TECHNIQUES; LIPOPROTEINS; LIVER; MALE; MICE; MICE, INBRED BALB C; MICE, MUTANT STRAINS; NIEMANN-PICK DISEASES; PROTEINS;

EID: 0036789434     PISSN: 02709139     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0270-9139(02)00088-5     Document Type: Article

References (44)

1. Pentchev PG, Vanier MT, Suzuki K, Patterson MC. Niemann-Pick disease Type C: a cellular cholesterol lipidosis. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular bases of Inherited Disease. Volume 2. New York: McGraw-Hill, 1995;2625-2640.
2. Liscum L, Klasek JJ. Niemann-Pick disease type C. Curr Opin Lipidol 1998;9:131-135.
3. Pentchev PG, Kruth HS, Comly ME, Butler JD, Vanier MT, Wenger DA, Patel S. Type C Niemann-Pick disease. A parallel loss of regulatory responses in the uptake and sterification of low-density lipoprotein-derived cholesterol cultured fibroblasts. J Biol Chem 1986;26:16775-16780.
4. Liscum L, Faust JR. Low density lipoprotein (LDL)-mediated suppression of cholesterol synthesis and LDL uptake is defective in Niemann-Pick type C fibroblasts. J Biol Chem 1987;262:17002-17008.
5. Blanchette-Mackie EJ, Dwyer NK, Amende LM, Kruth HS, Butler JD, Sokol J, Comly ME, et al. Type-C Niemann-Pick disease: low density lipoprotein uptake is associated with premature cholesterol accumulation in the Golgi complex and excessive cholesterol storage in lysosomes. Proc Natl Acad Sci U S A 1988;85:8022-8026.
6. Pentchev PG, Brady RO, Blanchette-Mackie EJ, Varnier MT, Cartsea ED, Parker CC, Godin E, et al. The Niemann-Pick C lesion and its relationship to the intracellular distribution and utilization of LDL Cholesterol. Bio chim Biophys Acta. 1994;1225:235-243.
7. Cartsea ED, Morris JA, Coleman JK, Loftus SK, Zhang D, Cummings C, Gu J, et al. Niemann-Pick C1 disease gene: homology to mediators to cholesterol homeostasis. Science 1997;277:228-231.
8. Neufeld EB, Patel S, SureshS, CooneyAM, Dwyer NK, Roff CF, et al. The Niemann-Pick C protein resides in a vesicular compartment linked to retrograde transport for multiple lysosomal cargo. J Biol Chem 1999;274:9627-9635.
9. Millard EE, Srivastava K, Traub LM, Schaffer JE, Ory DS. NPCI overexpression alters cellular cholesterol homeostasis. J Biol Chem 2000;275:38445-38451.
10. Pentchev PG, Boothe AD, Kruth HS, Weintroub H, Stivers J, Brady RO. A genetic storage disorder in BALB/c mice with a metabolic block in sterification of exogenous cholesterol. J Biol Chem 1984;259:5784-5791.
11. Loftus SK, Morris JA, Cattsea ED, Gu JZ, Cummings C, Brown A, Ellison J, et al. Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene. Science 1997;277:232-235.
12. Xie C, Turley SD, Pentchev PG, Dietschy JM. Cholesterol balance and metabolism in mice with loss of function of Niemann-Pick C protein. Am J Physiol 1999;276:E336-E334.
13. Xie C, Turley SD, Dietschy JM. Cholesterol accumulation in tissues of the Niemann-Pick type C mouse is determined by the rate of lipoproteincholesterol uptake through the coated-pit pathway in each organ. Proc Natl Acad Sci U S A 1999;96:1992-11997.
14. Xie C, Turley SD, Dietschy JM. Centripetal cholesterol flow from the extrahepatic organs through the liver is normal in mice with mutated Niemann-Pick type C protein. J Lipid Res 2000;41:1278-1289.
15. Dietschy JM, Turley SD. Control of cholesterol turnover in the mouse. J Biol Chem 2002;277:3801-3804.
16. Dietschy JM, Turley SD, Spady DK. Role of the liver in the maintenance of cholesterol and low density lipoprotein homeosrasis in different animal species, including humans. J Lipid Res 1993;34:1637-1659.
17. Turley SD, Dietschy JM. The metabolism and excretion of cholesterol by the liver. In: Arias IM, Jakoby WB, Popper H, Schachter D, Shafritz DA, eds. The Liver: biology and pathology. New York: Raven Press, 1988;617-641.
18. Liscum L, Munn NJ. Intracellular cholesterol transport. Biochim Biophys Acta 1999;1438:19-37.
19. Cohen DE. Hepatocellular transport and secretion of biliary lipids. Curr Opin Lipidol 1999;10:295-302.
20. He T-C, Zhou S, da Costa LT, Yu J, Kinzler KW, Vogelstein B. A symplified system for generating recombinant adenovirues. Proc Nad Acad Sci U S A 1998;95:2509-2514.
21. Kozarsky KF, Jooss K, Donahee M, Strauss JF, Wilson JM. Effective treatment of familial hypercholesterolaemia in the mouse model using adenovirus-mediated transfer of the VLDL receptor gene. Nat Genet 1996;10:54-62.
22. Chomczynski P, Sacchi N. Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction. Anal Biochem 1987;162:156-159.
23. Zanlungo S, Amigo L, Mendoza H, Miquel JF, Vio C, Glick JM, Rodriguez A, et al. Overexpression ofsterol carrier protein-2 leads to altered lipid metabolism and enhanced enterohepatic sterol circulation in mice. Gastroenterology 2000;119:1708-1719.
24. Mardones P, Quiñiones V, Amigo L, Moreno M, Miquel JF, Schwarz M, Miettinen HE, et al. Hepatic cholesterol and bile acid metabolism and intestinal cholesterol absorption in scavenger receptor class B type I-deficient mice. J Lipid Res 2001;42:170-180.
25. Jokinen EV, Landschulz KT, Wyne KL, Ho YK, Frykman PK, Hobbs HH. Regulation of very low density lipoprotein receptor by thyroid hormone in rat skeletal muscle. J Biol Chem 1994;269:26411-26418.
26. Rigotti A, Trigatti BL, Penman M, Rayburn HM, Herz J, Krieger MA. A targeted mutation in the murine gene encoding the high density lipoprorein (HDL) receptor scavenger receptor class B type I reveals its key role in HDL metabolism. Proc Nad Acad Sci U S A 1997;94:12610-12615.
27. Nervi F, Del Pozo R, Covarrubias C, Ronco B. The effect of progesterone on the regulatory mechanisms of biliary cholesterol secretion in the rat. Hepatology 1983;3:360-367.
28. Nervi F, Marinovic I, Rigotti A, Ulloa N. Regulation of biliary cholesterol secretion. Functional relationship between the canalicular and sinusoidal cholesterol secretory pathways in the rat. J Clin Invest 1988;82:1818-1825.
29. Haghpassand M, Bourasa P-AK, Francone OL, Aiello R.J. Monocyte/macrophage expression of ABCA 1 has minimal contribution to plasma HDL levels. J Clin Invest 2001;108:1315-1320.
30. Baez JM, Barbow SE, Cohen DE. Phosphatidylcholine transfer protein promotes apolipoprotein A-I mediated lipid efflux in Chinese hamster ovary cells. J Biol Chem 2002;277:6198-6206.
31. Berge KE, Tian H, Graf GA, Yu L, Grishin NV, Schultz J, Kwirerovich P, et al. Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters. Science 2001;290:1771-1775.
32. Repa JJ, Berge KE, Pomajzl C, Richardson JA, Hobbs HH, Mangelsdorf DJ. Regulation of ATP-binding cassette sterol transporters, by the oxysterol receptors, LXRα and β. J Biol Chem 2002;277:18793-18800.
33. Garver WS, Randall AH, Erickson RP, Thomas MA, Wilson JM. Localization of the murine Niemann-Pick C1 protein to two distinct intracellular compartments. J Lipid Res 2000;41:673-687.
34. Vaisman BL, Lambert G, Amar M, Joyce C, Ito T, Shamburek RD, Cain WJ, et al. ABCAI overexpression leads to hyperalphalipoproteinernia and increased biliary cholesterol excretion in transgenic mice. J Clin Invest 2001;108:303-309.
35. Singaraja RR, Bocher V, James ER, Clee SM, Zhang L-H, Leavitt BR, Tan B, et al. Human ABCA1 BAC transgenic mice show increased high density lipoprotein cholesterol and apoAI-dependent effllux stimulated by an internal promoter containg liver X receptor response elements in intron 1.J Biol Chem 2001;276:33969-33979.
36. Venkateswaran A, Laffitte BA, Joseph SB, Mak PA, Wilpitz DC, Edwards PA, Tontonoz P. Control of cellular cholesterol efflux by the nuclear oxysterol receptor LXRα. Proc Natl Acad Sci U S A 2000;97:12097-12102.
37. Shamburek RD, Pentchev PG, Zech LA, Blanchette-Mackie J, Carstea ED, VandenBroek JM, Cooper PS, et al. Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation. J Lipid Res 1997;38:2422-2435.
38. Chen W, Sun Y, Welch C, Gorelik A, Leventhal AR, Tabas I, Tall AR. Preferential ATP-Binding cassette transporter A1 (ABCAI)-mediated cholesterol efflux from late endosomes/lysosomes. J Biol Chem 2001;276:43364-43369.
39. Alpy F, Stoeckel AF, Dierich A, Escola JM, Wendling C, Chenard MP, Vanier M, et al. The steroidogenis acute regulatory protein homolog MLN64, a late endosomal cholesterol-binding protein. J Biol Chem 2001;276:4261-4269.
40. Fielding CJ, Fielding PE. Intracellular cholesterol transport. J Lipid Res 1997;38:1503-1521.
41. Garver WS, Krishnan K, Gallagos JR, Michikawa M, Francis GA, Heidenreich RA. Niemann-Pick C1 protein regulates cholesterol transport to the trans-Golgi network and plasma membrane caveolae. J Lipid Res 2002;43:579-589.
42. Garver WS, Erickson RP, Wilson JM, Colton TL, Hossain GS, Kosloski MA, Heidenreich RA. Altered expression of caveolin-1 and increased cholesterol in detergent insoluble membrane fractions from liver in mice with Niemann-Pick disease type C. Biochim Biophys Acta 1997;1361:272-280.
43. Amigo L, Quifiones V, Mardones P, Zanlungo S, Miquel JF, Nervi F, Rigotti A. Impaired biliary cholesterol secretion and decreased gallstone formation in apolipoprotein E-deficient mice fed a high-cholesterol diet. Gastroenterology 2000;118:772-779.
44. Lammert F, Carey MC, Paigen B. Chromosomal organization of candidate genes involved in cholesterol gallstone formation: a murinegallstone map. Gastroenterology 2001;120:221-238.