Cholesterol sensing, trafficking, and esterification

Annual Review of Cell and Developmental Biology

Volumn 22, Issue , 2006, Pages 129-157

  Chang, Ta Yuan ^a   Chang, Catherine C Y ^a   Ohgami, Nobutaka ^b   Yamauchi, Yoshio ^a  

^a DARTMOUTH MEDICAL SCHOOL   (United States)

^b CHUBU UNIVERSITY   (Japan)

Author keywords

Cholesterol efflux; Cholesterol recycling; Cholesteryl esters; Lipid rafts; Membrane trafficking; Nonvesicular lipid trafficking

Indexed keywords

ACYLTRANSFERASE; CHOLESTEROL; CHOLESTEROL ACYLTRANSFERASE; CHOLESTEROL ESTER; HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE; LIPID; LOW DENSITY LIPOPROTEIN; MEMBRANE PROTEIN; NIEMANN PICK TYPE C1 PROTEIN; OXYSTEROL; OXYSTEROL BINDING PROTEIN RELATED PROTEIN; PROTEIN; PROTEIN ACAT1; PROTEIN NPC1; STEROL REGULATORY ELEMENT BINDING PROTEIN; UNCLASSIFIED DRUG;

BIOSYNTHESIS; CELL MEMBRANE; CHOLESTEROL SYNTHESIS; EFFUSION; ENDOPLASMIC RETICULUM; ENDOSOME; ESTERIFICATION; HUMAN; LIPID ANALYSIS; LIPID TRANSPORT; MAMMAL CELL; NONHUMAN; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN DOMAIN; PROTEIN FUNCTION; PROTEIN MOTIF; PROTEIN STRUCTURE; REVIEW; STEROL SENSING DOMAIN;

AMINO ACID SEQUENCE; ANIMALS; BIOLOGICAL TRANSPORT; CHOLESTEROL; ESTERIFICATION; HUMANS; MEMBRANE GLYCOPROTEINS; MOLECULAR SEQUENCE DATA; PREGNENOLONE;

MAMMALIA;

EID: 33749073795     PISSN: 10810706     EISSN: None     Source Type: Book Series    
DOI: 10.1146/annurev.cellbio.22.010305.104656     Document Type: Review

References (145)

1. Adams CM, Reitz J, De Brabander JK, Feramisco JD, Li L, et al. 2004. Cholesterol and 25-hydroxycholesterol inhibit activation of SREBPs by different mechanisms, both involving SCAP and Insigs. J. Biol. Chem. 279:52772-80
2. Alberts B, Johnson AE, Lewis J, Raff M, Roberts K, Walter P. 2002. Molecular Biology of the Cell. New York: Garland Sci. 4th ed.
3. Alpy F, Tomasetto C. 2005. Give lipids a START: the StAR-related lipid transfer (START) domain in mammals. J. Cell Sci. 118:2791-801
4. Azhar S, Reaven E. 2002. Scavenger receptor class BI and selective cholesteryl partners in the regulation of steroidogenesis. Mol. Cell. Endocrinol. 195:1-26
5. Beh CT, Rine J. 2004. A role for yeast oxysterol-binding protein homologs in endocytosis and in the maintenance of intracellular sterol-lipid distribution. J. Cell Sci. 117:2983-96
6. Bernard DW, Rodriguez A, Rothblat GH, Glick JM. 1990. Influence of high density lipoprotein on esterified cholesterol stores in macrophages and hepatoma cells. Arteriosclerosis 10:135-44
7. Brewer HBJ, Santamarina-Fojo S. 2003. Clinical significance of high-density lipoproteins and the development of atherosclerosis: focus on the role of the adenosine triphosphate-binding cassette protein A1 transporter. Am. J. Cardiol. 92:10k-16k
8. Brown AJ, Jessup W. 1999. Oxysterols and atherosclerosis. Atherosclerosis 142:1-28
9. Brown AJ, Sun L, Feramisco JD, Brown MS, Goldstein JL. 2002. Cholesterol addition to ER membranes alters conformation of SCAP, the SREBP escort protein that regulates cholesterol metabolism. Mol. Cell 10:237-45
10. Brown DA, London E. 1998. Functions of lipid rafts in biological membranes. Annu. Rev. Cell Dev. Biol. 14:111-36
11. Brown MS, Dana S, Goldstein JL. 1975. Receptor-dependent hydrolysis of cholesteryl esters contained in plasma low density lipoprotein. Proc. Natl. Acad. Sci. USA 72:2925-29
12. Brown MS, Goldstein JL. 1986. A receptor-mediated pathway for cholesterol homeostasis. Science 232:34-47
13. Brown MS, Ho YK, Goldstein JL. 1980. The cholesteryl ester cycle in macrophage foam cells. J. Biol. Chem. 255:9344-52
14. Cadigan KM, Spillane DM, Chang TY. 1990. Isolation and characterization of Chinese hamster ovary cell mutants defective in intracellular low density lipoprotein-cholesterol trafficking. J. Cell Biol. 110:295-308
15. Carstea ED, Morris JA, Coleman KG, Loftus SK, Zhang D, et al. 1997. Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science 277:228-31
16. Cases S, Smith SJ, Zheng YW, Myers HM, Lear SR, et al. 1998. Identification of a gene encoding an acyl CoA:diacylglycerol acyltransferase, a key enzyme in triacylglycerol synthesis. Proc. Natl. Acad. Sci. USA 95:13018-23
17. Chang CCY, Lee CYG, Chang ET, Cruz JC, Levesque MC, Chang TY. 1998. Recombinant human acyl-CoA:cholesterol acyltransferase-1 (ACAT-1) purified to essential homogeneity utilizes cholesterol in mixed micelles or vesicles in a highly cooperative manner. J. Biol. Chem. 273:35132-41
18. Chang CCY, Sakashita N, Ornvold K, Lee O, Chang ET, et al. 2000. Immunological quantitation and localization of ACAT-1 and ACAT-2 in human liver and small intestine. J. Biol. Chem. 275:28083-92
19. Chang TY, Chang CCY, Cheng D. 1997. Acyl-coenzyme A:cholesterol acyltransferase. Annu. Rev. Biochem. 66:613-38
20. Chang TY, Chang CCY, Lin S, Yu C, Li BL, Miyazaki A. 2001. Roles of acyl-coenzyme A:cholesterol acyltransferase-1 and -2. Curr. Opin. Lipidol. 12:289-96
21. Chang TY, Limanek JS. 1980. Regulation of cytosolic acetoacetyl coenzyme A thiolase, 3-hydroxy-3-methylglutarylcoenzyme A synthase, 3-hydroxy-3- methylglutaryl coenzyme A reductase, and mevalonate kinase by low density lipoprotein and by 25-hydroxycholesteral in Chinese hamster ovary cells. J. Biol. Chem. 255:7787-95
22. Chang TY, Reid PC, Sugii S, Ohgami N, Cruz JC, Chang CCY. 2005. The Niemann-Pick type C disease and intracellular cholesterol trafficking. J. Biol. Chem. 280:20917-20
23. Chawla A, Repa JJ, Evans RM, Mangelsdorf DJ. 2001. Nuclear receptors and lipid physiology: opening the X-files. Science 294:1866-70
24. Chen FW, Gordon RE, Ioannou YA. 2005. NPC1 late endosomes contain elevated levels of nonesterified ('free') fatty acids and an abnormally glycosylated form of the NPC2 protein. Biochem. J. 390:549-61
25. Choudhury A, Dominguez M, Puri V, Sharma DK, Narita K, et al. 2002. Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells. J. Clin. Invest. 109:1541-50
26. Cohen AW, Hnasko R, Schubert W, Lisanti MP. 2004. Role of caveolae and caveolins in health and disease. Physiol. Rev. 84:1341-79
27. Compagnone NA, Mellon SH. 2000. Neurosteroids: biosynthesis and function of these novel neuromodulators. Front. Neuroendocrinol. 21:1-56
28. Cruz JC, Chang TY. 2000. Fate of endogenously synthesized cholesterol in Niemann-Pick type C1 cells. J. Biol. Chem. 275:41309-16
29. Cruz JC, Sugii S, Yu C, Chang TY. 2000. Role of Niemann-Pick type C1 protein in intracellular trafficking of low density lipoprotein-derived cholesterol. J. Biol. Chem. 275:4013-21
30. Davies JP, Chen FW, Ioannou YA. 2000. Transmembrane molecular pump activity of Niemann-Pick C1 protein. Science 290:2295-98
31. Davies JP, Ioannou YA. 2000. Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-activating protein. J. Biol. Chem. 275:24367-74
32. Dawson PA, Ridgway ND, Slaughter CA, Brown MS, Goldstein JL. 1989. cDNA cloning and expression of oxysterol-binding protein, an oligomer with a potential leucine zipper. J. Biol. Chem. 264:16798-803
33. Dean M, Annilo T. 2005. Evolution of the ATP-binding cassette (ABC) transporter superfamily in vertebrates. Annu. Rev. Genomics Hum. Genet. 6:123-42
34. Dietschy JM, Turley SD, Spady DK. 1993. Role of liver in the maintenance of cholesterol and low density lipoprotein homeostasis in different animal species, including humans. J. Lipid Res. 34:1637-59
35. Farese RVJ. 1998. Acyl-CoA:cholesterol acyltransferase genes and knockout mice. Curr. Opin. Lipidol. 9:119-24
36. Fielding CJ, Fielding PE. 2001. Cellular cholesterol efflux. Biochim. Biophys. Acta 1533:175-89
37. Friedland N, Liou HL, Lobel P, Stock AM. 2003. Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease. Proc. Natl. Acad. Sci. USA 100:2512-17
38. Frolov A, Zielinski SE, Crowley JR, Dudley-Rucker N, Schaffer JE, Ory DS. 2003. NPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterols. J. Biol. Chem. 278:25517-25
39. Fu X, Menke JG, Chen Y, Zhou G, MacNaul KL, et al. 2001. 27-hydroxycholesterol is an endogenous ligand for liver X receptor in cholesterol-loaded cells. J. Biol. Chem. 276:38378-87
40. Garcia-Calvo M, Lisnock J, Bull HG, Hawes BE, Burnett DA, et al. 2005. The target of ezetimibe is Niemann-Pick C1-like 1 (NPC1L1). Proc. Natl. Acad. Sci. USA 102:8132-37
41. Garver WS, Heidenreich RA, Erickson RP, Thomas MA, Wilson JM. 2000. Localization of the murine Niemann-Pick C1 protein to two distinct intracellular compartments. J. Lipid Res. 41:673-87
42. Goldstein JL, DeBose-Boyd RA, Brown MS. 2006. Protein sensors for membrane sterols. Cell 124:35-46
43. Griffin LD, Gong W, Verot L, Mellon SH. 2004. Niemann-Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone. Nat. Med. 10:704-11
44. Gruber A, Mancek M, Wagner H, Kirschning CJ, Jerala R. 2004. Structural model of MD-2 and functional role of its basic amino acid clusters involved in cellular lipopolysaccharide recognition. J. Biol. Chem. 279:28475-82
45. Guo ZY, Lin S, Keinen JA, Chang CC, Chang TY. 2005. The active site His-460 of human acylcoenzyme A:cholesterol acyltransferase 1 resides in a hitherto undisclosed transmembrane domain. J. Biol. Chem. 280:37814-26
46. Hanada K, Kumagai K, Yasuda S, Miura Y, Kawano M, et al. 2003. Molecular machinery for nonvesicular trafficking of ceramide. Nature 426:803-9
47. Hao M, Maxfield FR. 2000. Characterization of rapid membrane internalization and recycling. J. Biol. Chem. 275:15279-86
48. Hauet T, Yao ZX, Bose HS, Wall CT, Han Z, et al. 2005. Peripheral-type benzodiazepine receptor-mediated action of steroidogenic acute regulatory protein on cholesterol entry into Leydig cell mitochondria. Mol. Endocrinol. 19:540-54
49. Hofmann K. 2000. A superfamily of membrane-bound O-acyltransferases with implications for Wnt signaling. Trends Biochem. Sci. 25:111-12
50. Hua X, Nohturfft A, Goldstein JL, Brown MS. 1996. Sterol resistance in CHO cells traced to point mutation in SREBP cleavage-activating protein. Cell 87:415-26
51. Im YJ, Raychaudhuri S, Prinz WA, Hurley JH. 2005. Structural mechanism for sterol sensing and transport by OSBP-related proteins. Nature 437:154-58
52. Kennedy MA, Barrera GC, Nakamura K, Baldan A, Tarr P, et al. 2005. ABCG1 has a critical role in mediating cholesterol efflux to HDL and preventing cellular lipid accumulation. Cell Metab. 1:121-31
53. Klansek JJ, Warner GJ, Johnson WJ, Glick JM. 1996. Compartmental isolation of cholesterol participating in the cytoplasmic cholesteryl ester cycle in Chinese hamster ovary 25-RA cells J. Biol. Chem. 271:4923-29
54. Hucken J, Buchler C, Orso E, Kaminski W, Porsch-Ozcurumez M, et al. 2000. ABCG1 (ABC8), the human homolog of the Drosophila white gene, is a regulator of macrophage cholesterol and phospholipid transport. Proc. Natl. Acad. Sci. USA 97:817-22
55. Ko DC, Binkley J, Sidow A, Scott MP. 2003. The integrity of a cholesterol-binding pocket in Niemann-Pick C2 protein is necessary to control lysosome cholesterol levels. Proc. Natl. Acad. Sci. USA 100:2518-25
56. Ko DC, Gordon MD, Jin JY, Scott MP. 2001. Dynamic movements of organelles containing Niemann-Pick C1 protein: NPC1 involvement in late endocytic events. Mol. Biol. Cell 12:601-14
57. Ko DC, Milenkovic L, Beier SM, Manuel H, Buchanan J, Scott MP. 2005. Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease. PLoS Genet. 1:81-95
58. Krieger M. 1999. Charting the fate of the "good" cholesterol": identification and characterization of the high density lipoprotein receptor SR-BI. Annu. Rev. Biochem. 68:523-58
59. Krishnamurthy M, Higaki K, Tinkelenberg AH, Balderes DA, Almanzar-Paramio D, et al. 2004. Mutagenesis of the putative sterol-sensing domain of yeast Niemann Pick C-related protein reveals a primordial role in subcellular sphingolipid distribution. J. Cell Biol. 164:547-56
60. Lange Y, Ye J, Steck TL. 1998. Circulation of cholesterol between lysosomes and the plasma membrane. J. Biol. Chem. 273:18915-22
61. Lehto M, Hynynen R, Karjalainen K, Kuismanen E, Hyvarinen K, Olkkonen VM. 2005. Targeting of OSBP-related protein 3 (ORP3) to endoplasmic reticulum and plasma membrane is controlled by multiple determinants. Exp. Cell Res. 310:445-62
62. Li BL, Chang TY, Chen J, Chang CC, Zhao XN. 2006. Human ACAT1 gene expression and its involvement in the development of atherosclerosis. Future Cardiol. 2:93-99
63. Li H, Yao ZX, Degenhardt B, Teper G, Papadopoulos V. 2001. Cholesterol binding at the cholesterol recognition/interaction amino acid consensus (CRAC) of the peripheral-type benzodiazepine receptor and inhibition of steroidogenesis by an HIV TAT-CRAC peptide. Proc. Natl. Acad. Sci. USA 98:1267-72
64. Li Y, Prinz WA. 2004. ATP-binding cassette (ABC) transporters mediate nonvesicular, raft-modulated sterol movement from the plasma membrane to the endoplasmic reticulum. J. Biol. Chem. 279:45226-34
65. Libby P, Aikawa M. 2002. Stabilization of atherosclerotic plaques: new mechanisms and clinical targets. Nat. Med. 8:1257-62
66. Lin S, Lu X, Chang CCY, Chang TY. 2003. Human acyl-coenzyme A: cholesterol acyltransferase 2 (hACAT2) expressed in Chinese hamster ovary Cells: membrane topology and active site location. Mol. Biol. Cell 14:2447-60
67. Linsel-Nitschke P, Tall AR. 2005. HDL as a target in the treatment of atherosclerotic cardiovascular disease. Nat. Rev. Drug Discov. 4:193-205
68. Liscum L, Ruggiero RM, Faust JR. 1989. The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts. J. Cell Biol. 108:1625-36
69. Liu J, Chang CC, Westover EJ, Covey DF, Chang TY. 2005. Investigating the allosterism of acyl coenzyme A:cholesterol acyltransferase (ACAT) by using various sterols: in vitro and intact cell studies. Biochem. J. 391:389-97
70. Loftus SK, Morris JA, Carstea ED, Gu JZ, Cummings C, et al. 1997. Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene. Science 277:232-35
71. Lund EG, Xie C, Kotti T, Turley SD, Dietschy JM, Russell DW. 2003. Knockout of the cholesterol 24-hydroxylase gene in mice reveals a brain-specific mechanism of cholesterol turnover. J. Biol. Chem. 278:22980-88
72. Martin V, Carrillo G, Torroja C, Guerrero I. 2001. The sterol-sensing domain of Patched protein seems to control smoothened activity through Patched vesicular trafficking. Curr. Biol. 11:601-7
73. Matveev S, Li XL, Everson W, Smart E. 2001. The role of caveolae and caveolin in vesicle-dependent and vesicle-independent trafficking. Adv. Drug Deliv. Rev. 49:237-50
74. Millard EE, Gale SE, Dudley N, Zhang J, Schaffer JE, Ory DS. 2005. The sterol-sensing domain of the Niemann-Pick C1 (NPC1) protein regulates trafficking of low density lipoprotein cholesterol. J. Biol. Chem. 280:28581-90
75. Miller WL, Strauss JF. 1999. Molecular pathology and mechanism of action of the steroidogenic acute regulatory protein, StAR. J. Steroid Biochem. Mol. Biol. 69:131-41
76. Mukherjee S, Maxfield FR. 2004. Membrane domains. Annu. Rev. Cell Dev. Biol. 20:839-66
77. Munro S. 2003. Cell biology: earthworms and lipid couriers. Nature 426:775-76
78. Neufeld EB, Cooney AM, Pitha J, Dawidowicz EA, Dwyer NK, et al. 1996. Intracellular trafficking of cholesterol monitored with a cyclodextrin. J. Biol. Chem. 271:21604-13
79. Ohashi M, Miwako I, Nakamura K, Yamamoto A, Murata M, et al. 1999. An arrested late endosome-lysosome intermediate aggregate observed in a Chinese hamster ovary cell mutant isolated by novel three-step screening. J. Cell Sci. 112:1125-38
80. Ohashi M, Miwako I, Yamamoto A, Nagayama K. 2000. Arrested maturing multivesicular endosomes observed in a Chinese hamster ovary cell mutant, LEX2, isolated by repeated flow-cytometric cell sorting. J. Cell Sci. 113:2187-205
81. Ohgami N, Ko DC, Thomas M, Scott MP, Chang CC, Chang TY. 2004. Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain. Proc. Natl. Acad. Sci. USA 101:12473-78
82. Olkkonen VM. 2004. Oxysterol binding protein and its homologues: new regulatory factors involved in lipid metabolism. Curr. Opin. Lipidol. 15:321-27
83. Oram JF. 2002. ATP-binding cassette transporter A1 and cholesterol trafficking. Curr. Opin. Lipidol. 13:373-81
84. Papadopoulos V. 1993. Peripheral-type benzodiazepine/diazepam binding inhibitor receptor: biological role in steroidogenic cell function. Endocr. Rev. 14:222-40
85. Parini P, Davis M, Lada AT, Erickson SK, Wright TL, et al. 2004. ACAT2 is localized to hepatocytes and is the major cholesterol-esterifying enzyme in human liver. Circulation 110:2017-23
86. Patterson MC, Vanier MT, Suzuki K, Morris JA, Carstea E, et al. 2001. Niemann-Pick disease type C: a lipid trafficking disorder. In The Metabolic and Molecular Bases of Inherited Disease, ed. CR Scriver, AL Beaudet, WS Sly, D Valle, pp. 3611-33. New York: McGraw-Hill
87. Pelkmans L, Helenius A. 2003. Insider information: what viruses tell us about endocytosis. Curr. Opin. Cell Biol. 15:414-22
88. Pelkmans L, Kartenbeck J, Helenius A. 2001. Caveolar endocytosis of simian virus 40 reveals a new two-step vesicular-transport pathway to the ER. Nat. Cell Biol. 3:473-83
89. Pfeffer S, Aivazian D. 2004. Targeting Rab GTPases to distinct membrane compartments. Nat. Rev. Mol. Cell Biol. 5:886-96
90. Pike LJ. 2003. Lipid rafts: bringing order to chaos. J. Lipid Res. 44:655-67
91. Pike LJ. 2004. Lipid rafts: heterogeneity on the high seas. Biochem. J. 378:281-92
92. Pike LJ, Han X, Gross RW. 2005. Epidermal growth factor receptors are localized to lipid rafts that contain a balance of inner and outer leaflet lipids: a shotgun lipidomics study. J. Biol. Chem. 280:26796-804
93. Ponting CP, Aravind L. 1999. START: a lipid-binding domain in StAR, HD-ZIP and signaling proteins. Trends Biochem. Sci. 24:130-32
94. Radhakrishnan A, Sun LP, Kwon HJ, Brown MS, Goldstein JL. 2004. Direct binding of cholesterol to the purified membrane region of SCAP: mechanism for a sterol-sensing domain. Mol. Cell 15:259-68
95. Razani B, Woodman SE, Lisanti MP. 2002. Caveolae: from cell biology to animal physiology. Pharmacol. Rev. 54:431-67
96. Reid PC, Sakashita N, Sugii S, Ohno-Iwashita Y, Shimada Y, et al. 2004. A novel cholesterol stain reveals early neuronal cholesterol accumulation in the Niemann-Pick type C1 mouse brain. J. Lipid Res. 45:582-91
97. Reid PC, Sugii S, Chang TY. 2003. Trafficking defects in endogenously synthesized cholesterol in fibroblasts, macrophages, hepatocytes, and glial cells from Niemann-Pick type C1 mice. J. Lipid Res. 44:1010-19
98. Repa JJ, Mangelsdorf DJ. 2000. The role of orphan nuclear receptors in the regulation of cholesterol homeostasis. Annu. Rev. Cell Dev. Biol. 16:459-81
99. Ridgeway ND, Dawson PA, Ho YK, Brown MS, Goldstein JL. 1992. Translocation of oxysterol binding protein to Golgi apparatus triggered by ligand binding. J. Cell Biol. 116:307-19
100. Robel P, Young J, Corpechot C, Mayo W, Perche F, et al. 1995. Biosynthesis and assay of neurosteroids in rats and mice: functional correlates. J. Steroid Biochem. Mol. Biol. 53:355-60
101. Roitelman J, Olender EH, Bar-Nun S, Dunn WAJ, Simoni RD. 1992. Immunological evidence for eight spans in the membrane domains of HMG-CoA reductase: implications for enzyme degradation in the endoplasmic reticulum. J. Cell Biol. 117:959-73
102. Romanowski MJ, Soccio RE, Breslow JL, Burley SK. 2002. Crystal structure of the Mus musculus cholesterol-regulated START protein 4 (StarD4) containing a StAR-related lipid transfer domain. Proc. Natl. Acad. Sci. USA 99:6949-54
103. Rothblat GH, de la Llera-Moya M, Atger V, Kellner-Weibel G, Williams DL, Phillips MC. 1999. Cell cholesterol efflux: integration of old and new observations provides new insights. J. Lipid Res. 40:781-96
104. Rudel L, Lee R, Cockman T. 2001. Structure, function, and regulation of ACAT. Curr. Opin. Lipidol. 12:121-27
105. Russell DW. 2003. The enzymes, regulation, and genetics of bile acid synthesis. Annu. Rev. Biochem. 72:137-74
106. Schroeder F, Gallegos AM, Atshaves BP, Storey SM, McIntosh AL, et al. 2001. Recent advances in membrane microdomains: rafts, caveolae, and intracellular cholesterol trafficking. Exp. Biol. Med. 226:873-90
107. Sever N, Song BL, Yabe D, Goldstein JL, Brown MS, DeBose-Boyd RA. 2003a. Insig-dependent ubiquitination and degradation of mammalian 3-hydroxy-3- methylglutaryl-CoA reductase stimulated by sterols and geranylgeraniol. J. Biol. Chem. 278:52479-90
108. Sever N, Yang T, Brown MS, Goldstein JL, DeBose-Boyd RA. 2003b. Accelerated degradation of HMG CoA reductase mediated by binding of insig-1 to its sterol-sensing domain. Mol. Cell. 11:25-33
109. Shearer AG, Hampton RY. 2005. Lipid-mediated, reversible misfolding of a sterol-sensing domain protein. EMBO J. 24:149-59
110. Simons K, Ikonen E. 1997. Functional rafts in cell membranes. Nature 387:569-72
111. Skiba PJ, Zha X, Maxfield FR, Schissel SL, Tabas I. 1996. The distal pathway of lipoprotein-induced cholesterol esterification, but not sphingomyelinase-induced cholesterol esterification, is energy-dependent. J. Biol. Chem. 271:13392-400
112. Sleat DE, Wiseman JA, El-Banna M, Price SM, Verot L, et al. 2004. Genetic evidence for nonredundant functional cooperativity between NPC1 andNPC2 in lipid transport. Proc. Natl. Acad. Sci. USA 101:5886-91
113. Slotte JP, Bierman EL. 1988. Depletion of plasma-membrane sphingomyelin rapidly alters the distribution of cholesterol between plasma membranes and intracellular cholesterol pool in cultured fibroblasts. Biochem. J. 250:653-58
114. Soccio RE, Breslow JL. 2003. StAR-related lipid transfer (START) proteins: mediators of intracellular lipid metabolism. J. Biol. Chem. 278:22183-86
115. Song BL, Wang CH, Yao XM, Yang L, Zhang WJ, et al. 2006. Human acyl-CoA: cholesterol acyltransferase 2 gene expression in intestinal Caco-2 cells and in hepatocellular carcinoma. Biochem. J. 394(Pt. 3):617-26
116. Stocco DM. 2001. Tracking the role of a star in the sky of the new millennium. Mol. Endocrinol. 15:1245-54
117. Strutt H, Thomas C, Nakano Y, Stark D, Neave B, et al. 2001. Mutations in the sterol-sensing domain of Patched suggest a role for vesicular trafficking in Smoothened regulation. Curr. Biol. 11:608-13
118. Sugii S, Reid PC, Ohgami N, Du H, Chang TY. 2003. Distinct endosomal compartments in early trafficking of low density lipoprotein-derived cholesterol. J. Biol. Chem. 278:27180-89
119. Tabas I. 2002. Consequences of cellular cholesterol accumulation: basic concepts and physiological implications. J. Clin. Invest. 110:905-11
120. Tabas I, Rosoff WJ, Boykow GC. 1988. Acyl coenzyme A:cholesterol acyl transferase in macrophages utilizes a cellular pool of cholesterol oxidase-accessible cholesterol as substrate. J. Biol. Chem. 263:1266-72
121. Taylor FR, Saucier SE, Shown EP, Parish EJ, Kandutsch AA. 1984. Correlation between oxysterol binding to a cytosolic binding protein and potency in the repression of hydroxymethylglutaryl coenzyme A reductase. J. Biol. Chem. 259:12382-87
122. Tsujishita Y, Hurley JH. 2000. Structure and lipid transport mechanism of a StAR-related domain. Nat. Struct. Biol. 7:408-14
123. Underwood KW, Jacobs NL, Howley A, Liscum L. 1998. Evidence for a cholesterol transport pathway from lysosomes to endoplasmic reticulum that is independent of the plasma membrane. J. Biol. Chem. 273:4266-74
124. Vainio S, Jansen M, Koivusalo M, Rog T, Karttunen M, et al. 2006. Significance of sterol structural specificity: Desmosterol cannot replace cholesterol in lipid rafts. J. Biol. Chem. 281:348-55
125. Vance JE, Hayashi H, Karten B. 2005. Cholesterol homeostasis in neurons and glial cells. Semin. Cell Dev. Biol. 16:193-212
126. Venkateswaran A, Laffitte BA, Joseph SB, Mak PA, Wilpitz DC, et al. 2000. Control of cellular cholesterol efflux by the nuclear oxysterol receptor LXRα. Proc. Natl. Acad. Sci. USA 97:12097-102
127. Walter M, Davies JP, Ioannou YA. 2003. Telomerase immortalization upregulates Rab9 expression and restores LDL cholesterol egress from Niemann-Pick C1 late endosomes. J. Lipid Res. 44:243-53
128. Wang N, Lan D, Chen W, Matsuura F, Tall AR. 2004. ATP-binding cassette transporters G1 and G4 mediate cellular cholesterol efflux to high-density lipoproteins. Proc. Natl. Acad. Sci. USA 101:9774-79
129. Wang P, Zhang Y, Li H, Chieu HK, Munn AL, Yang H. 2005a. AAA ATPases regulate membrane association of yeast oxysterol binding proteins and sterol metabolism. EMBO J. 24:2989-99
130. Wang PY, Weng J, Anderson RG. 2005b. OSBP is a cholesterol-regulated scaffolding protein in control of ERK 1/2 activation. Science 307:1472-76
131. Watari H, Blanchette-Mackie EJ, Dwyer NK, Watari M, Burd CG, et al. 2000. Determinants of NPC1 expression and action: key promoter regions, posttranscriptional control, and the importance of a "cysteine-rich" loop. Exp. Cell Res. 259:247-56
132. Westover EJ, Covey DF. 2004. The enantiomer of cholesterol. J. Membr. Biol. 202:61-72
133. Willenborg M, Schmidt CK, Braun P, Landgrebe J, van Figura K, et al. 2005. Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation. J. Lipid Res. 46:2559-69
134. Wojtanik KM, Liscum L. 2003. The transport of LDL-derived cholesterol to the plasma membrane is defective in NPC1 cells. J. Biol. Chem. 278:14850-56
135. Wyles JP, Ridgeway ND. 2004. VAMP-associated protein-A regulates partitioning of oxysterol-binding protein-related protein-9 between the endoplasmic reticulum and Golgi apparatus. Exp. Cell Res. 297:533-47
136. Xu L, Simoni RD. 2003. The inhibition of degradation of 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase by sterol regulatory element binding protein cleavage-activating protein requires four phenylalanine residues in span 6 of HMG-CoA reductase transmembrane domain. Arch. Biochem. Biophys. 414:232-43
137. Yamauchi Y, Chang CC, Hayashi M, Abe-Dohmae S, Reid PC, et al. 2004. Intracellular cholesterol mobilization involved in the ABCA1/apolipoprotein- mediated assembly of high density lipoprotein in fibroblasts. J. Lipid Res. 45:1943-51
138. Yang T, Espenshade PJ, Wright ME, Yabe D, Gong Y, et al. 2002. Crucial step in cholesterol homeostasis: sterols promote binding of SCAP to INSIG-1, a membrane protein that facilitates retention of SREBPs in ER. Cell 110:489-500
139. Yokoyama S. 2005. Assembly of high density lipoprotein by the ABCA1/apolipoprotein pathway. Curr. Opin. Lipidol. 16:269-79
140. Yu C, Chen J, Lin S, Liu J, Chang CCY, Chang TY. 1999. Human acyl-CoA:cholesterol acyltransferase-1 is a homotetrameric enzyme in intact cells and in vitro. J. Biol. Chem. 274:36139-45
141. Yu C, Zhang Y, Lu X, Chang CCY, Chang TY. 2002. The role of the N-terminal hydrophilic domain of acyl coenzyme A:cholesterol acyltransferase 1 on the enzyme's quaternary structure and catalytic efficiency. Biochemistry 41:3762-69
142. Yu L, Hammer R, Li-Hawkins J, Bergmann K, Lutjohann D, et al. 2002. Disruption of Abcg5 and Abcg8 in mice reveals their crucial role in biliary cholesterol secretion. Proc. Natl. Acad. Sci. USA 99:16237-42
143. Yu W, Gong JS, Ko M, Garver WS, Yanagisawa K, Michikawa M. 2005. Altered cholesterol metabolism in Niemann-Pick type C1 mouse brains affects mitochondrial function. J. Biol. Chem. 280:11731-39
144. Zervas M, Somers KL, Thrall MA, Walkley SU. 2001. Critical role of glycosphingolipids in Niemann-Pick disease type C. Curr. Biol. 11:1283-87
145. Zhang Y, Yu C, Liu J, Spencer TA, Chang CC, Chang TY. 2003. Cholesterol is superior to 7-ketocholesterol or 7α-hydroxycholesterol as an allosteric activator for acyl-coenzyme A: cholesterol acyltransferase 1. J. Biol. Chem. 278:11642-47