Cholesterol homeostasis in neurons and glial cells

Seminars in Cell and Developmental Biology

Volumn 16, Issue 2, 2005, Pages 193-212

  Vance, Jean E ^a   Hayashi, Hideki ^a,b   Karten, Barbara ^a,b  

^a UNIVERSITY OF ALBERTA   (Canada)

^b ALBERTA ENVIRONMENT AND PARKS   (Canada)

Author keywords

Alzheimer's disease; Cholesterol; Glia; Neurons; Niemann Pick C disease

Indexed keywords

24 HYDROXYCHOLESTEROL; 3ALPHA HYDROXY 5ALPHA PREGNAN 20 ONE; 3BETA (2 DIETHYLAMINOETHOXY)ANDROST 5 EN 17 ONE; APOLIPOPROTEIN E; CHOLESTEROL; CHOLESTEROL ACYLTRANSFERASE INHIBITOR; CHOLINESTERASE INHIBITOR; CLUSTERIN; DONEPEZIL; HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE INHIBITOR; MEVINOLIN; MIGLUSTAT; N METHYL DEXTRO ASPARTIC ACID RECEPTOR BLOCKING AGENT; PRAVASTATIN; ROSCOVITINE; SIMVASTATIN; UNCLASSIFIED DRUG;

ALZHEIMER DISEASE; BLOOD BRAIN BARRIER; CENTRAL NERVOUS SYSTEM; CHOLESTEROL METABOLISM; CHOLESTEROL SYNTHESIS; CHOLESTEROL TRANSPORT; CLINICAL TRIAL; DOSE RESPONSE; DRUG EFFECT; GLIA CELL; HOMEOSTASIS; HUMAN; HYPERCHOLESTEROLEMIA; LIPOPROTEIN BLOOD LEVEL; MOTOR DYSFUNCTION; NERVE CELL; NIEMANN PICK DISEASE; NONHUMAN; PROTEIN SECRETION; REVIEW; SENILE PLAQUE; TURNOVER TIME;

EID: 15944389528     PISSN: 10849521     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.semcdb.2005.01.005     Document Type: Review

References (277)

1. J.M. Dietschy, and S.D. Turley Thematic review series: brain lipids. Cholesterol metabolism in the central nervous system during early development and in the mature animal J Lipid Res 45 2004 1375 1397
2. Y. Lange, M.H. Swaisgood, B.V. Ramos, and T.L. Steck Plasma membranes contain half the phospholipid and 90% of the cholesterol and sphingomyelin in cultured human fibroblasts J Biol Chem 264 1989 3786 3793
3. D.K. Spady, and J.M. Dietschy Sterol synthesis in vivo in 18 tissues of the squirrel monkey, guinea pig, rabbit, hamster, and rat J Lipid Res 24 1983 303 315
4. 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
5. J.M. Dietschy, and S.D. Turley Control of cholesterol turnover in the mouse J Biol Chem 277 2002 3801 3804
6. S. Acton, A. Rigotti, K.T. Landschulz, S. Xu, H.H. Hobbs, and M. Krieger Identification of scavenger receptor SR-BI as a high density lipoprotein receptor Science 271 1996 518 520
7. H. Jurevics, and P. Morell Cholesterol for synthesis of myelin is made locally, not imported into brain J Neurochem 64 1995 895 901
8. S.D. Turley, D.K. Burns, and J.M. Dietschy Preferential utilization of newly synthesized cholesterol for brain growth in neonatal lambs Am J Physiol 274 1998 1099 1105
9. S.D. Turley, D.K. Burns, C.R. Rosenfeld, and J.M. Dietschy Brain does not utilize low density lipoprotein-cholesterol during fetal and neonatal development in the sheep J Lipid Res 37 1996 1953 1961
10. Y. Osono, L.A. Woollett, J. Herz, and J.M. Dietschy Role of the low density lipoprotein receptor in the flux of cholesterol through the plasma and across the tissues of the mouse J Clin Invest 95 1995 1124 1132
11. J.M. Dietschy, T. Kita, K.E. Suckling, J.L. Goldstein, and M.S. Brown Cholesterol synthesis in vivo and in vitro in the WHHL rabbit, an animal with defective low density lipoprotein receptors J Lipid Res 24 1983 469 480
12. L. Yu, K. von Bergmann, D. Lutjohann, H.H. Hobbs, and J.C. Cohen Selective sterol accumulation in ABCG5/ABCG8-deficient mice J Lipid Res 45 2004 301 307
13. G. Salen, I. Horak, M. Rothkopf, J.L. Cohen, J. Speck, and G.S. Tint Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis J Lipid Res 26 1985 1126 1133
14. G. Quan, C. Xie, J.M. Dietschy, and S.D. Turley Ontogenesis and regulation of cholesterol metabolism in the central nervous system of the mouse Brain Res Dev Brain Res 146 2003 87 98
15. U. Panzenboeck, Z. Balazs, A. Sovic, A. Hrzenjak, S. Levak-Frank, and A. Wintersperger ABCA1 and scavenger receptor class B, type I, are modulators of reverse sterol transport at an in vitro blood-brain barrier constituted of porcine brain capillary endothelial cells J Biol Chem 277 2002 42781 42789
16. Z. Balazs, U. Panzenboeck, A. Hammer, A. Sovic, O. Quehenberger, and E. Malle Uptake and transport of high-density lipoprotein (HDL) and HDL-associated alpha-tocopherol by an in vitro blood-brain barrier model J Neurochem 89 2004 939 950
17. I. Bjorkhem, D. Lutjohann, O. Breuer, A. Sakinis, and A. Wennmalm Importance of a novel oxidative mechanism for elimination of brain cholesterol turnover of cholesterol and 24(S)-hydroxycholesterol in rat brain as measured with 18O2 techniques in vivo and in vitro J Biol Chem 272 1997 30178 30184
18. E.G. Lund, C. Xie, T. Kotti, S.D. Turley, J.M. Dietschy, and D.W. Russell Knockout of the cholesterol 24-hydroxylase gene in mice reveals a brain-specific mechanism of cholesterol turnover J Biol Chem 278 2003 22980 22988
19. E.G. Lund, J.M. Guileyardo, and D.W. Russell cDNA cloning of cholesterol 24-hydroxylase, a mediator of cholesterol homeostasis in the brain Proc Natl Acad Sci USA 96 1999 7238 7243
20. D. Lutjohann, O. Breuer, G. Ahlborg, I. Nennesmo, A. Siden, and U. Diczfalusy Cholesterol homeostasis in human brain: evidence for an age-dependent flux of 24S-hydroxycholesterol from the brain into the circulation Proc Natl Acad Sci USA 93 1996 9799 9804
21. Y. Lange, J. Ye, and F. Strebel Movement of 25-hydroxycholesterol from the plasma membrane to the rough endoplasmic reticulum in cultured hepatoma cells J Lipid Res 36 1995 1092 1097
22. S. Meaney, K. Bodin, U. Diczfalusy, and I. Bjorkhem On the rate of translocation in vitro and kinetics in vivo of the major oxysterols in human circulation: critical importance of the position of the oxygen function J Lipid Res 43 2002 2130 2135
23. I. Bjorkhem, D. Lutjohann, U. Diczfalusy, L. Stahle, G. Ahlborg, and J. Wahren Cholesterol homeostasis in human brain: turnover of 24S- hydroxycholesterol and evidence for a cerebral origin of most of this oxysterol in the circulation J Lipid Res 39 1998 1594 1600
24. N. Wang, D. Lan, W. Chen, F. Matsuura, and A.R. Tall ATP-binding cassette transporters G1 and G4 mediate cellular cholesterol efflux to high-density lipoproteins Proc Natl Acad Sci USA 101 2004 9774 9779
25. K. Nakamura, M.A. Kennedy, A. Baldan, D.D. Bojanic, K. Lyons, and P.A. Edwards Expression and regulation of multiple murine ATP-binding cassette transporter G1 mRNAs/isoforms that stimulate cellular cholesterol efflux to high density lipoprotein J Biol Chem 279 2004 45980 45989
26. M.J. LaDu, J.A. Shah, C.A. Reardon, G.S. Getz, G. Bu, and J. Hu Apolipoprotein E receptors mediate the effects of beta-amyloid on astrocyte cultures J Biol Chem 275 2000 33974 33980
27. R.E. Pitas, J.K. Boyles, S.H. Lee, D. Hui, and K.H. Weisgraber Lipoproteins and their receptors in the central nervous system. Characterization of the lipoproteins in cerebrospinal fluid and identification of apolipoprotein B,E(LDL) receptors in the brain J Biol Chem 262 1987 14352 14360
28. M.J. LaDu, S.M. Gilligan, J.R. Lukens, V.G. Cabana, C.A. Reardon, and L.J. Van Eldik Nascent astrocyte particles differ from lipoproteins in CSF J Neurochem 70 1998 2070 2081
29. S. Koch, N. Donarski, K. Goetze, M. Kreckel, H.J. Stuerenburg, and C. Buhmann Characterization of four lipoprotein classes in human cerebrospinal fluid J Lipid Res 42 2001 1143 1151
30. J.S. Gong, M. Kobayashi, H. Hayashi, K. Zou, N. Sawamura, and S.C. Fujita Apolipoprotein E (ApoE) isoform-dependent lipid release from astrocytes prepared from human ApoE3 and ApoE4 knock-in mice J Biol Chem 277 2002 29919 29926
31. W.J. Brecht, F.M. Harris, S. Chang, I. Tesseur, G.Q. Yu, and Q. Xu Neuron-specific apolipoprotein e4 proteolysis is associated with increased tau phosphorylation in brains of transgenic mice J Neurosci 24 2004 2527 2534
32. T. Rothe, and H.W. Muller Uptake of endoneurial lipoprotein into Schwann cells and sensory neurons is mediated by low density lipoprotein receptors and stimulated after axonal injury J Neurochem 57 1991 2016 2025
33. E.I. Posse De Chaves, D.E. Vance, R.B. Campenot, R.S. Kiss, and J.E. Vance Uptake of lipoproteins for axonal growth of sympathetic neurons J Biol Chem 275 2000 19883 19890
34. U. Beffert, P.C. Stolt, and J. Herz Functions of lipoprotein receptors in neurons J Lipid Res 45 2004 403 409
35. M. Trommsdorff, J.-P. Borg, B. Margolis, and J. Herz Interaction of cytosolic adaptor proteins with neuronal apolipoprotein E receptors and the amyloid precursor protein J Biol Chem 273 1998 33556 33560
36. M. Trommsdorff, M. Gotthardt, T. Hiesberger, J. Shelton, W. Stockinger, and J. Nimpf Reeler/disabled-like disruption of neuronal migration in knockout mice lacking the VLDL receptor and apoE receptor 2 Cell 97 1999 689 701
37. K.H. Weisgraber Apolipoprotein E: structure-function relationships Adv Protein Chem 45 1994 249 302
38. E.H. Corder, A.M. Saunders, W.J. Strittmatter, D.E. Schmechel, P.C. Gaskell, and G.W. Small Gene dose of apolipoprotein E type 4 allele and the risk of Alzheimer's disease in late onset families Science 261 1993 921 923
39. W.J. Strittmatter, K.H. Weisgraber, D.Y. Huang, L.M. Dong, G.S. Salvesen, and M. Pericak-Vance Binding of human apolipoprotein E to synthetic amyloid beta peptide: isoform-specific effects and implications for late-onset Alzheimer disease Proc Natl Acad Sci USA 90 1993 8098 8102
40. J. Poirier, J. Davignon, D. Bouthillier, S. Kogan, P. Bertrand, and S. Gauthier Apolipoprotein E polymorphism and Alzheimer's disease Lancet 342 1993 697 699
41. M.J. Ignatius, P.J. Gebicke-Harter, J.H. Skene, J.W. Schilling, K.H. Weisgraber, and R.W. Mahley Expression of apolipoprotein E during nerve degeneration and regeneration Proc Natl Acad Sci USA 83 1986 1125 1129
42. J.H.P. Skene, and E.M. Shooter Denervated sheath cells secrete a new protein after nerve injury Proc Natl Acad Sci USA 80 1986 4169 4173
43. J.K. Boyles, C.D. Zoellner, L.J. Anderson, L.M. Kosik, R.E. Pitas, and K.H. Weisgraber A role for apolipoprotein E, apolipoprotein A-I, and low density lipoprotein receptors in cholesterol transport during regeneration and remyelination of the rat sciatic nerve J Clin Invest 83 1989 1015 1031
44. R.W. Mahley Apolipoprotein E: cholesterol transport protein with expanding role in cell biology Science 240 1988 622 630
45. A.M. Fagan, B.A. Murphy, S.N. Patel, J.F. Kilbridge, W.C. Mobley, and G. Bu Evidence for normal aging of the septo-hippocampal cholinergic system in apoE (-/-) mice but impaired clearance of axonal degeneration products following injury Exp Neurol 151 1998 314 325
46. B.P. Nathan, S. Bellosta, D.A. Sanan, K.H. Weisgraber, R.W. Mahley, and R.E. Pitas Differential effects of apolipoproteins E3 and E4 on neuronal growth in vitro Science 264 1994 850 852
47. S. Bellosta, B. Nathan, M. Orth, L.-M. Dong, R.W. Mahley, and R.E. Pitas Stable expression and secretion of apolipoproteins E3 and E4 in mouse neuroblastoma cells produces differential effects on neurite outgrowth J Biol Chem 270 1995 27063 27071
48. R.B. DeMattos, L.K. Curtiss, and D.L. Williams A minimally lipidated form of cell-derived apolipoprotein E exhibits isoform-specific stimulation of neurite outgrowth in the absence of exogenous lipids or lipoproteins J Biol Chem 273 1998 4206 4212
49. J.E. Vance, D. Pan, R.B. Campenot, M. Bussiere, and D.E. Vance Evidence that the major membrane lipids, except cholesterol, are made in axons of cultured rat sympathetic neurons J Neurochem 62 1994 329 337
50. R.B. Campenot Independent control of the local environment of somas and neurites Methods Enzymol 28 1979 302 307
51. R.B. Campenot Development of sympathetic neurons in compartmentalized cultures: I. Local control of neurite growth by nerve growth factor Dev Biol 93 1982 1 12
52. E.I. Posse de Chaves, A.E. Rusiñol, D.E. Vance, R.B. Campenot, and J.E. Vance Role of lipoproteins in the delivery of lipids to axons during axonal regeneration J Biol Chem 272 1997 30766 30773
53. E.I. Posse de Chaves, D.E. Vance, R.B. Campenot, R.S. Kiss, and J.E. Vance Uptake of lipoproteins for axonal growth of sympathetic neurons J Biol Chem 275 2000 19883 19890
54. H. Hayashi, R.B. Campenot, D.E. Vance, and J.E. Vance Glial lipoproteins stimulate axon growth of central nervous system neurons in compartmented cultures J Biol Chem 279 2004 14009 14015
55. D.H. Mauch, K. Nagler, S. Schumacher, C. Goritz, E.C. Muller, and A. Otto CNS synaptogenesis promoted by glia-derived cholesterol Science 294 2001 1354 1357
56. S.N. Romas, M.X. Tang, L. Berglund, and R. Mayeux APOE genotype, plasma lipids, lipoproteins, and AD in community elderly Neurology 53 1999 517 521
57. G.P. Jarvik, E.M. Wijsman, W.A. Kukull, G.D. Schellenberg, C. Yu, and E.B. Larson Interactions of apolipoprotein E genotype, total cholesterol level, age, and sex in prediction of Alzheimer's disease: a case-control study Neurology 45 1995 1092 1096
58. I.L. Notkola, R. Sulkava, J. Pekkanen, T. Erkinjuntti, C. Ehnholm, and P. Kivinen Serum total cholesterol, apolipoprotein E epsilon 4 allele, and Alzheimer's disease Neuroepidemiology 17 1998 14 20
59. R.M. Evans, C.L. Emsley, S. Gao, A. Sahota, K.S. Hall, and M.R. Farlow Serum cholesterol, APOE genotype, and the risk of Alzheimer's disease: a population-based study of African Americans Neurology 54 2000 240 242
60. M. Kivipelto, E.L. Helkala, M.P. Laakso, T. Hanninen, M. Hallikainen, and K. Alhainen Apolipoprotein E epsilon4 allele, elevated midlife total cholesterol level, and high midlife systolic blood pressure are independent risk factors for late-life Alzheimer disease Ann Intern Med 137 2002 149 155
61. L. Svennerholm, K. Bostrom, B. Jungbjer, and L. Olsson Membrane lipids of adult human brain: lipid composition of frontal and temporal lobe in subjects of age 20 to 100 years J Neurochem 63 1994 1802 1811
62. L. Svennerholm, and C.G. Gottfries Membrane lipids, selectively diminished in Alzheimer brains, suggest synapse loss as a primary event in early-onset form (type I) and demyelination in late-onset form (type II) J Neurochem 62 1994 1039 1047
63. Z.S. Tan, S. Seshadri, A. Beiser, P.W. Wilson, D.P. Kiel, and M. Tocco Plasma total cholesterol level as a risk factor for Alzheimer disease: the Framingham Study Arch Intern Med 163 2003 1053 1057
64. H. Jick, G.L. Zornberg, S.S. Jick, S. Seshadri, and D.A. Drachman Statins and the risk of dementia Lancet 356 2000 1627 1631
65. B. Wolozin, W. Kellman, P. Ruosseau, G.G. Celesia, and G. Siegel Decreased prevalence of Alzheimer disease associated with 3-hydroxy-3- methyglutaryl coenzyme A reductase inhibitors Arch Neurol 57 2000 1439 1443
66. D.S. Howland, S.P. Trusko, M.J. Savage, A.G. Reaume, D.M. Lang, and J.D. Hirsch Modulation of secreted beta-amyloid precursor protein and amyloid beta-peptide in brain by cholesterol J Biol Chem 273 1998 16576 16582
67. L.M. Refolo, B. Malester, J. LaFrancois, T. Bryant-Thomas, R. Wang, and G.S. Tint Hypercholesterolemia accelerates the Alzheimer's amyloid pathology in a transgenic mouse model Neurobiol Dis 7 2000 321 331
68. F.S. Shie, L.W. Jin, D.G. Cook, J.B. Leverenz, and R.C. LeBoeuf Diet-induced hypercholesterolemia enhances brain A beta accumulation in transgenic mice Neuroreport 13 2002 455 459
69. M. Crisby, S.M. Rahman, C. Sylven, B. Winblad, and M. Schultzberg Effects of high cholesterol diet on gliosis in apolipoprotein E knockout mice. Implications for Alzheimer's disease and stroke Neurosci Lett 369 2004 87 92
70. D. Lutjohann, A. Papassotiropoulos, I. Bjorkhem, S. Locatelli, M. Bagli, and R.D. Oehring Plasma 24S-hydroxycholesterol (cerebrosterol) is increased in Alzheimer and vascular demented patients J Lipid Res 41 2000 195 198
71. A. Papassotiropoulos, D. Lutjohann, M. Bagli, S. Locatelli, F. Jessen, and R. Buschfort 24S-Hydroxycholesterol in cerebrospinal fluid is elevated in early stages of dementia J Psychiatr Res 36 2002 27 32
72. P. Schonknecht, D. Lutjohann, J. Pantel, H. Bardenheuer, T. Hartmann, and K. von Bergmann Cerebrospinal fluid 24S-hydroxycholesterol is increased in patients with Alzheimer's disease compared to healthy controls Neurosci Lett 324 2002 83 85
73. L. Bretillon, A. Siden, L.O. Wahlund, D. Lutjohann, L. Minthon, and M. Crisby Plasma levels of 24S-hydroxycholesterol in patients with neurological diseases Neurosci Lett 293 2000 87 90
74. M. Heverin, N. Bogdanovic, D. Lutjohann, T. Bayer, I. Pikuleva, and L. Bretillon Changes in the levels of cerebral and extracerebral sterols in the brain of patients with Alzheimer's disease J Lipid Res 45 2004 186 193
75. I. Bjorkhem, and S. Meaney Brain cholesterol: long secret life behind a barrier Arterioscler Thromb Vasc Biol 24 2004 806 815
76. D.J. Selkoe Alzheimer's disease: genes, proteins, and therapy Physiol Rev 81 2001 741 766
77. B. Wolozin Cholesterol and the biology of Alzheimer's disease Neuron 41 2004 7 10
78. M. Simons, P. Keller, J. Dichgans, and J.B. Schulz Cholesterol and Alzheimer's disease: is there a link? Neurology 57 2001 1089 1093
79. D.J. Selkoe Alzheimer disease: mechanistic understanding predicts novel therapies Ann Intern Med 140 2004 627 638
80. T. Mori, D. Paris, T. Town, A.M. Rojiani, D.L. Sparks, and A. Delledonne Cholesterol accumulates in senile plaques of Alzheimer disease patients and in transgenic APP(SW) mice J Neuropathol Exp Neurol 60 2001 778 785
81. M. Simons, P. Keller, B. De Strooper, K. Beyreuther, C.G. Dotti, and K. Simons Cholesterol depletion inhibits the generation of beta-amyloid in hippocampal neurons Proc Natl Acad Sci USA 95 1998 6460 6464
82. E. Kojro, G. Gimpl, S. Lammich, W. Marz, and F. Fahrenholz Low cholesterol stimulates the nonamyloidogenic pathway by its effect on the alpha-secretase ADAM 10 Proc Natl Acad Sci USA 98 2001 5815 5820
83. L. Marlow, M. Cain, M.A. Pappolla, and K. Sambamurti Beta-secretase processing of the Alzheimer's amyloid protein precursor (APP) J Mol Neurosci 20 2003 233 239
84. K.S. Vetrivel, H. Cheng, W. Lin, T. Sakurai, T. Li, and N. Nukina Association of gamma-secretase with lipid rafts in post-Golgi and endosome membranes J Biol Chem 279 2004 44945 44954
85. R. Ehehalt, P. Keller, C. Haass, C. Thiele, and K. Simons Amyloidogenic processing of the Alzheimer beta-amyloid precursor protein depends on lipid rafts J Cell Biol 160 2003 113 123
86. S. Wada, M. Morishima-Kawashima, Y. Qi, H. Misono, Y. Shimada, and Y. Ohno-Iwashita Gamma-secretase activity is present in rafts but is not cholesterol-dependent Biochemistry 42 2003 13977 13986
87. J. Brown III, C. Theisler, S. Silberman, D. Magnuson, N. Gottardi-Littell, and J.M. Lee Differential expression of cholesterol hydroxylases in Alzheimer's disease J Biol Chem 279 2004 34674 34681
88. M. Michikawa, and K. Yanagisawa Apolipoprotein E4 induces neuronal cell death under conditions of suppressed de novo cholesterol synthesis J Neurosci Res 54 1998 58 67
89. M.J. LaDu, M.T. Falduto, A.M. Manelli, C.A. Reardon, G.S. Getz, and D.E. Frail Isoform-specific binding of apolipoprotein E to beta-amyloid J Biol Chem 269 1994 23403 23406
90. W.J. Strittmatter, K.H. Weisgraber, M. Goedert, A.M. Saunders, D. Huang, and E.H. Corder Hypothesis: microtubule instability and paired helical filament formation in the Alzheimer disease brain are related to apolipoprotein E genotype Exp Neurol 125 1994 163 171 [discussion 172-64]
91. M. Miyata, and J.D. Smith Apolipoprotein E allele-specific antioxidant activity and effects on cytotoxicity by oxidative insults and beta-amyloid peptides Nat Genet 14 1996 55 61
92. D.E. Schmechel, A.M. Saunders, W.J. Strittmatter, B.J. Crain, C.M. Hulette, and S.H. Joo Increased amyloid beta-peptide deposition in cerebral cortex as a consequence of apolipoprotein E genotype in late-onset Alzheimer disease Proc Natl Acad Sci USA 90 1993 9649 9653
93. A.D. Roses Apolipoprotein E alleles as risk factors in Alzheimer's disease Annu Rev Med 47 1996 387 400
94. A.M. Fagan, M. Watson, M. Parsadanian, K.R. Bales, S.M. Paul, and D.M. Holtzman Human and murine ApoE markedly alters A beta metabolism before and after plaque formation in a mouse model of Alzheimer's disease Neurobiol Dis 9 2002 305 318
95. J.A. Prince, H. Zetterberg, N. Andreasen, J. Marcusson, and K. Blennow APOE epsilon4 allele is associated with reduced cerebrospinal fluid levels of Abeta42 Neurology 62 2004 2116 2118
96. R.B. DeMattos Apolipoprotein E dose-dependent modulation of beta-amyloid deposition in a transgenic mouse model of Alzheimer's disease J Mol Neurosci 23 2004 255 262
97. K.M. Mann, F.E. Thorngate, Y. Katoh-Fukui, H. Hamanaka, D.L. Williams, and S. Fujita Independent effects of APOE on cholesterol metabolism and brain Abeta levels in an Alzheimer disease mouse model Hum Mol Genet 13 2004 1959 1968
98. A.M. Fagan, E. Christopher, J.W. Taylor, M. Parsadanian, M. Spinner, and M. Watson ApoAI deficiency results in marked reductions in plasma cholesterol but no alterations in amyloid-beta pathology in a mouse model of Alzheimer's disease-like cerebral amyloidosis Am J Pathol 165 2004 1413 1422
99. Y. Huang, X.Q. Liu, T. Wyss-Coray, W.J. Brecht, D.A. Sanan, and R.W. Mahley Apolipoprotein E fragments present in Alzheimer's disease brains induce neurofibrillary tangle-like intracellular inclusions in neurons Proc Natl Acad Sci USA 98 2001 8838 8843
100. F.M. Harris, W.J. Brecht, Q. Xu, I. Tesseur, L. Kekonius, and T. Wyss-Coray Carboxyl-terminal-truncated apolipoprotein E4 causes Alzheimer's disease-like neurodegeneration and behavioral deficits in transgenic mice Proc Natl Acad Sci USA 100 2003 10966 10971
101. W. Gibson Wood, G.P. Eckert, U. Igbavboa, and W.E. Muller Amyloid beta-protein interactions with membranes and cholesterol: causes or casualties of Alzheimer's disease Biochim Biophys Acta 1610 2003 281 290
102. U. Igbavboa, N.A. Avdulov, F. Schroeder, and W.G. Wood Increasing age alters transbilayer fluidity and cholesterol asymmetry in synaptic plasma membranes of mice J Neurochem 66 1996 1717 1725
103. H. Hayashi, U. Igbavboa, H. Hamanaka, M. Kobayashi, S.C. Fujita, and W.G. Wood Cholesterol is increased in the exofacial leaflet of synaptic plasma membranes of human apolipoprotein E4 knock-in mice Neuroreport 13 2002 383 386
104. N.B. Chauhan Membrane dynamics, cholesterol homeostasis, and Alzheimer's disease J Lipid Res 44 2003 2019 2029
105. A. Kakio, S.I. Nishimoto, K. Yanagisawa, Y. Kozutsumi, and K. Matsuzaki Cholesterol-dependent formation of GM1 ganglioside-bound amyloid beta-protein, an endogenous seed for Alzheimer amyloid J Biol Chem 276 2001 24985 24990
106. K. Yanagisawa, A. Odaka, N. Suzuki, and Y. Ihara GM1 ganglioside-bound amyloid beta-protein (A beta): a possible form of preamyloid in Alzheimer's disease Nat Med 1 1995 1062 1066
107. G.M. Pasinetti, S.A. Johnson, T. Oda, I. Rozovsky, and C.E. Finch Clusterin (SGP-2): a multifunctional glycoprotein with regional expression in astrocytes and neurons of the adult rat brain J Comp Neurol 339 1994 387 400
108. E. Matsubara, J. Ghiso, B. Frangione, M. Amari, Y. Tomidokoro, and Y. Ikeda Lipoprotein-free amyloidogenic peptides in plasma are elevated in patients with sporadic Alzheimer's disease and Down's syndrome Ann Neurol 45 1999 537 541
109. J. Ghiso, E. Matsubara, A. Koudinov, N.H. Choi-Miura, M. Tomita, and T. Wisniewski The cerebrospinal-fluid soluble form of Alzheimer's amyloid beta is complexed to SP-40,40 (apolipoprotein J), an inhibitor of the complement membrane-attack complex Biochem J 293 Pt 1 1993 27 30
110. E. Matsubara, B. Frangione, and J. Ghiso Characterization of apolipoprotein J-Alzheimer's A beta interaction J Biol Chem 270 1995 7563 7567
111. P. Strocchi, F. Rauzi, and D. Cevolani Neuronal loss up-regulates clusterin mRNA in living neurons and glial cells in the rat brain Neuroreport 10 1999 1789 1792
112. F. White, J.A. Nicoll, and K. Horsburgh Alterations in ApoE and ApoJ in relation to degeneration and regeneration in a mouse model of entorhinal cortex lesion Exp Neurol 169 2001 307 318
113. E. Matsubara, C. Soto, S. Governale, B. Frangione, and J. Ghiso Apolipoprotein J and Alzheimer's amyloid beta solubility Biochem J 316 1996 671 679
114. L.N. Boggs, K.S. Fuson, M. Baez, L. Churgay, D. McClure, and G. Becker Clusterin (Apo J) protects against in vitro amyloid-beta (1-40) neurotoxicity J Neurochem 67 1996 1324 1327
115. B.V. Zlokovic, C.L. Martel, J.B. Mackic, E. Matsubara, T. Wisniewski, and J.G. McComb Brain uptake of circulating apolipoproteins J and E complexed to Alzheimer's amyloid beta Biochem Biophys Res Commun 205 1994 1431 1437
116. B.V. Zlokovic, C.L. Martel, E. Matsubara, J.G. McComb, G. Zheng, and R.T. McCluskey Glycoprotein 330/megalin: probable role in receptor-mediated transport of apolipoprotein J alone and in a complex with Alzheimer disease amyloid beta at the blood-brain and blood-cerebrospinal fluid barriers Proc Natl Acad Sci USA 93 1996 4229 4234
117. S.M. Hammad, S. Ranganathan, E. Loukinova, W.O. Twal, and W.S. Argraves Interaction of apolipoprotein J-amyloid beta-peptide complex with low density lipoprotein receptor-related protein-2/megalin. A mechanism to prevent pathological accumulation of amyloid beta-peptide J Biol Chem 272 1997 18644 18649
118. P. Giannakopoulos, E. Kovari, L.E. French, I. Viard, P.R. Hof, and C. Bouras Possible neuroprotective role of clusterin in Alzheimer's disease: a quantitative immunocytochemical study Acta Neuropathol (Berl) 95 1998 387 394
119. P.C. May, M. Lampert-Etchells, S.A. Johnson, J. Poirier, J.N. Masters, and C.E. Finch Dynamics of gene expression for a hippocampal glycoprotein elevated in Alzheimer's disease and in response to experimental lesions in rat Neuron 5 1990 831 839
120. T. Oda, G.M. Pasinetti, H.H. Osterburg, C. Anderson, S.A. Johnson, and C.E. Finch Purification and characterization of brain clusterin Biochem Biophys Res Commun 204 1994 1131 1136
121. N.H. Choi-Miura, Y. Ihara, K. Fukuchi, M. Takeda, Y. Nakano, and T. Tobe SP-40, 40 is a constituent of Alzheimer's amyloid Acta Neuropathol (Berl) 83 1992 260 264
122. A.M. Lidstrom, N. Bogdanovic, C. Hesse, I. Volkman, P. Davidsson, and K. Blennow Clusterin (apolipoprotein J) protein levels are increased in hippocampus and in frontal cortex in Alzheimer's disease Exp Neurol 154 1998 511 521
123. A.M. Lidstrom, C. Hesse, L. Rosengren, P. Fredman, P. Davidsson, and K. Blennow Normal levels of clusterin in cerebrospinal fluid in Alzheimer's disease, and no change after acute ischemic stroke J Alzheimer's Dis 3 2001 435 442
124. B. Tycko, L. Feng, L. Nguyen, A. Francis, A. Hays, and W.Y. Chung Polymorphisms in the human apolipoprotein-J/clusterin gene: ethnic variation and distribution in Alzheimer's disease Hum Genet 98 1996 430 436
125. R.B. DeMattos, M.A. O'Dell, M. Parsadanian, J.W. Taylor, J.A. Harmony, and K.R. Bales Clusterin promotes amyloid plaque formation and is critical for neuritic toxicity in a mouse model of Alzheimer's disease Proc Natl Acad Sci USA 99 2002 10843 10848
126. B.H. Han, R.B. DeMattos, L.L. Dugan, J.S. Kim-Han, R.P. Brendza, and J.D. Fryer Clusterin contributes to caspase-3-independent brain injury following neonatal hypoxia-ischemia Nat Med 7 2001 338 343
127. K. Fassbender, M. Simons, C. Bergmann, M. Stroick, D. Lutjohann, and P. Keller Simvastatin strongly reduces levels of Alzheimer's disease beta-amyloid peptides Abeta 42 and Abeta 40 in vitro and in vivo Proc Natl Acad Sci USA 98 2001 5856 5861
128. S. Parvathy, M. Ehrlich, S. Pedrini, N. Diaz, L. Refolo, and J.D. Buxbaum Atorvastatin-induced activation of Alzheimer's alpha secretase is resistant to standard inhibitors of protein phosphorylation-regulated ectodomain shedding J Neurochem 90 2004 1005 1010
129. N.B. Chauhan, G.J. Siegel, and D.L. Feinstein Effects of lovastatin and pravastatin on amyloid processing and inflammatory response in TgCRND8 brain Neurochem Res 29 2004 1897 1911
130. G.L. Vega, M.F. Weiner, A.M. Lipton, K. Von Bergmann, D. Lutjohann, and C. Moore Reduction in levels of 24S-hydroxycholesterol by statin treatment in patients with Alzheimer disease Arch Neurol 60 2003 510 515
131. B. Borroni, C. Pettenati, T. Bordonali, N. Akkawi, M. Di Luca, and A. Padovani Serum cholesterol levels modulate long-term efficacy of cholinesterase inhibitors in Alzheimer disease Neurosci Lett 343 2003 213 215
132. A. Adunsky, V. Chesnin, R. Ravona, D. Harats, and M. Davidson Plasma lipid levels in Alzheimer's disease patients treated by Donepezil hydrochloride: a cross-sectional study Arch Gerontol Geriatr 38 2004 61 68
133. J. Shepherd, G.J. Blauw, M.B. Murphy, E.L. Bollen, B.M. Buckley, and S.M. Cobbe Pravastatin in elderly individuals at risk of vascular disease (PROSPER): a randomised controlled trial Lancet 360 2002 1623 1630
134. MRC/BHF Heart Protection Study of cholesterol lowering with simvastatin in 20, 536 high-risk individuals: a randomised placebo-controlled trial. Lancet 2002;360:7-22.
135. B. Wolozin Cholesterol, statins and dementia Curr Opin Lipidol 15 2004 667 672
136. I. Casserly, and E. Topol Convergence of atherosclerosis and Alzheimer's disease: inflammation, cholesterol, and misfolded proteins Lancet 363 2004 1139 1146
137. K. Hoglund, O. Wiklund, H. Vanderstichele, O. Eikenberg, E. Vanmechelen, and K. Blennow Plasma levels of beta-amyloid (1-40), beta-amyloid (1-42), and total beta-amyloid remain unaffected in adult patients with hypercholesterolemia after treatment with statins Arch Neurol 61 2004 333 337
138. K. Fassbender, M. Stroick, T. Bertsch, A. Ragoschke, S. Kuehl, and S. Walter Effects of statins on human cerebral cholesterol metabolism and secretion of Alzheimer amyloid peptide Neurology 59 2002 1257 1258
139. J. Triscari, J.S. Markowitz, and M.W. McGovern Pravastatin and lovastatin in cerebrospinal fluid Clin Neuropharmacol 16 1993 559 560
140. H. Lennernas, and G. Fager Pharmacodynamics and pharmacokinetics of the HMG-CoA reductase inhibitors. Similarities and differences Clin Pharmacokinet 32 1997 403 425
141. K. Pahan, F.G. Sheikh, A.M. Namboodiri, and I. Singh Lovastatin and phenylacetate inhibit the induction of nitric oxide synthase and cytokines in rat primary astrocytes, microglia, and macrophages J Clin Invest 100 1997 2671 2679
142. S. Amin-Hanjani, N.E. Stagliano, M. Yamada, P.L. Huang, J.K. Liao, and M.A. Moskowitz Mevastatin, an HMG-CoA reductase inhibitor, reduces stroke damage and upregulates endothelial nitric oxide synthase in mice Stroke 32 2001 980 986
143. C.J. Vaughan, and A.M. Gotto Jr. Update on statins: 2003 Circulation 110 2004 886 892
144. L. Puglielli, G. Konopka, E. Pack-Chung, L.A. Ingano, O. Berezovska, and B.T. Hyman Acyl-coenzyme A: cholesterol acyltransferase modulates the generation of the amyloid beta-peptide Nat Cell Biol 3 2001 905 912
145. B. Hutter-Paier, H.J. Huttunen, L. Puglielli, C.B. Eckman, D.Y. Kim, and A. Hofmeister The ACAT inhibitor CP-113,818 markedly reduces amyloid pathology in a mouse model of Alzheimer's disease Neuron 44 2004 227 238
146. M.A. Wollmer, J.R. Streffer, M. Tsolaki, L.M. Grimaldi, D. Lutjohann, and D. Thal Genetic association of acyl-coenzyme A: cholesterol acyltransferase with cerebrospinal fluid cholesterol levels, brain amyloid load, and risk for Alzheimer's disease Mol Psychiatry 8 2003 635 638
147. H. Kolsch, D. Lutjohann, M. Ludwig, A. Schulte, U. Ptok, and F. Jessen Polymorphism in the cholesterol 24S-hydroxylase gene is associated with Alzheimer's disease Mol Psychiatry 7 2002 899 902
148. A. Johansson, H. Katzov, H. Zetterberg, L. Feuk, B. Johansson, and N. Bogdanovic Variants of CYP46A1 may interact with age and APOE to influence CSF Abeta42 levels in Alzheimer's disease Hum Genet 114 2004 581 587
149. A. Papassotiropoulos, J.R. Streffer, M. Tsolaki, S. Schmid, D. Thal, and F. Nicosia Increased brain beta-amyloid load, phosphorylated tau, and risk of Alzheimer disease associated with an intronic CYP46 polymorphism Arch Neurol 60 2003 29 35
150. B. Borroni, S. Archetti, C. Agosti, N. Akkawi, C. Brambilla, and L. Caimi Intronic CYP46 polymorphism along with ApoE genotype in sporadic Alzheimer disease: from risk factors to disease modulators Neurobiol Aging 25 2004 747 751
151. J.F. Oram, and R.M. Lawn ABCA1. The gatekeeper for eliminating excess tissue cholesterol J Lipid Res 42 2001 1173 1179
152. J.J. Repa, S.D. Turley, J.A. Lobaccaro, J. Medina, L. Li, and K. Lustig Regulation of absorption and ABC1-mediated efflux of cholesterol by RXR heterodimers Science 289 2000 1524 1529
153. S.E. Wahrle, H. Jiang, M. Parsadanian, J. Legleiter, X. Han, and J.D. Fryer ABCA1 is required for normal central nervous system ApoE levels and for lipidation of astrocyte-secreted apoE J Biol Chem 279 2004 40987 40993
154. M.A. Wollmer, J.R. Streffer, D. Lutjohann, M. Tsolaki, V. Iakovidou, and T. Hegi ABCA1 modulates CSF cholesterol levels and influences the age at onset of Alzheimer's disease Neurobiol Aging 24 2003 421 426
155. H. Katzov, K. Chalmers, J. Palmgren, N. Andreasen, B. Johansson, and N.J. Cairns Genetic variants of ABCA1 modify Alzheimer disease risk and quantitative traits related to beta-amyloid metabolism Hum Mutat 23 2004 358 367
156. L. Terrisse, J. Poirier, P. Bertrand, A. Merched, S. Visvikis, and G. Siest Increased levels of apolipoprotein D in cerebrospinal fluid and hippocampus of Alzheimer's patients J Neurochem 71 1998 1643 1650
157. B. Belloir, E. Kovari, M. Surini-Demiri, and A. Savioz Altered apolipoprotein D expression in the brain of patients with Alzheimer disease J Neurosci Res 64 2001 61 69
158. E.A. Thomas, L.N. Sautkulis, J.R. Criado, D. Games, and J.G. Sutcliffe Apolipoprotein D mRNA expression is elevated in PDAPP transgenic mice J Neurochem 79 2001 1059 1064
159. A. Navarro, E. Del Valle, A. Astudillo, C. Gonzalez del Rey, and J. Tolivia Immunohistochemical study of distribution of apolipoproteins E and D in human cerebral beta amyloid deposits Exp Neurol 184 2003 697 704
160. P.P. Desai, H.C. Hendrie, R.M. Evans, J.R. Murrell, S.T. DeKosky, and M.I. Kamboh Genetic variation in apolipoprotein D affects the risk of Alzheimer disease in African-Americans Am J Med Genet 116B 2003 98 101
161. S. Helisalmi, M. Hiltunen, S. Vepsalainen, S. Iivonen, E.H. Corder, and M. Lehtovirta Genetic variation in apolipoprotein D and Alzheimer's disease J Neurol 251 2004 951 957
162. D.E. Kang, C.U. Pietrzik, L. Baum, N. Chevallier, D.E. Merriam, and M.Z. Kounnas Modulation of amyloid beta-protein clearance and Alzheimer's disease susceptibility by the LDL receptor-related protein pathway J Clin Invest 106 2000 1159 1166
163. M. Shibata, S. Yamada, S.R. Kumar, M. Calero, J. Bading, and B. Frangione Clearance of Alzheimer's amyloid-ss(1-40) peptide from brain by LDL receptor-related protein-1 at the blood-brain barrier J Clin Invest 106 2000 1489 1499
164. J.C. Lambert, F. Wavrant-De Vrieze, P. Amouyel, and M.C. Chartier-Harlin Association at LRP gene locus with sporadic late-onset Alzheimer's disease Lancet 351 1998 1787 1788
165. L. Baum, L. Chen, H.K. Ng, Y.S. Chan, Y.T. Mak, and J. Woo Low density lipoprotein receptor related protein gene exon 3 polymorphism association with Alzheimer's disease in Chinese Neurosci Lett 247 1998 33 36
166. H. Kolsch, U. Ptok, I. Mohamed, S. Schmitz, M.L. Rao, and W. Maier Association of the C766T polymorphism of the low-density lipoprotein receptor-related protein gene with Alzheimer's disease Am J Med Genet 121B 2003 128 130
167. E. Hollenbach, S. Ackermann, B.T. Hyman, and G.W. Rebeck Confirmation of an association between a polymorphism in exon 3 of the low-density lipoprotein receptor-related protein gene and Alzheimer's disease Neurology 50 1998 1905 1907
168. M. Sanchez-Guerra, O. Combarros, J. Infante, J. Llorca, J. Berciano, and A. Fontalba Case-control study and meta-analysis of low density lipoprotein receptor-related protein gene exon 3 polymorphism in Alzheimer's disease Neurosci Lett 316 2001 17 20
169. J.K. Fink, M.R. Filling-Katz, J. Sokol, D.G. Cogan, A. Pikus, and B. Sonies Clinical spectrum of Niemann-Pick disease type C Neurology 39 1989 1040 1049
170. P.G. Pentchev, M.T. Vanier, K. Suzuki, and M.C. Patterson Niemann-Pick Disease type C: a cellular cholesterol lipidosis C.R. Scriver A.L. Beaudet W.S. Sly D. Valle The metabolic and molecular basis of inherited disease vol. II 1995 McGraw-Hill New York 2625 2639
171. I. Vincent, B. Bu, and R.P. Erickson Understanding Niemann-Pick type C disease: a fat problem Curr Opin Neurol 16 2003 155 161
172. S.L. Sturley, M.C. Patterson, W. Balch, and L. Liscum The pathophysiology and mechanisms of NP-C disease Biochim Biophys Acta 1685 2004 83 87
173. J. Sokol, E.J. Blanchette-Mackie, H.S. Kruth, N.K. Dwyer, L.M. Amende, and J.D. Butler Type C Niemann-Pick Disease. Lysosomal accumulation and defective intracellular mobilization of low density lipoprotein cholesterol J Biol Chem 263 1988 3411 3417
174. T. Kobayashi, M.H. Beuchat, M. Lindsay, S. Frias, R.D. Palmiter, and H. Sakuraba Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport Nat Cell Biol 1 1999 113 118
175. M.T. Vanier, R. Rousson, and P. Louisot Chromatofocusing of skin fibroblast sphingomyelinase: alterations in Niemann-Pick disease type C shared by GM1-gangliosidosis Clin Chim Acta 130 1983 155 161
176. M. Zervas, K.L. Somers, M.A. Thrall, and S.U. Walkley Critical role for glycosphingolipids in Niemann-Pick disease type C Curr Biol 11 2001 1283 1287
177. Y. Watanabe, S. Akaboshi, G. Ishida, T. Takeshima, T. Yano, and M. Taniguchi Increased levels of GM2 ganglioside in fibroblasts from a patient with juvenile Niemann-Pick disease type C Brain Dev 20 1998 95 97
178. E. Goldin, C.F. Roff, S.P.F. Miller, C. Rodriguez-Lafrasse, M.T. Vanier, and R.O. Brady Type C Niemann-Pick disease: a murine model of the lysosomal cholesterol lipidosis accumulates sphingosine and sphinganine in liver Biochim Biophys Acta 1127 1992 303 311
179. M.C. Patterson, and F. Platt Therapy of Niemann-Pick disease, type C Biochim Biophys Acta 1685 2004 77 82
180. S. Miyawaki, H. Yoshida, S. Mitsuoka, H. Enomoto, and S. Ikehara A mouse model for Niemann-Pick disease. Influence of genetic background on disease expression in spm/spm mice J Hered 77 1986 379 384
181. S.K. Loftus, J.A. Morris, E.D. Carstea, J.Z. Gu, C. Cummings, and A. Brown Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene Science 277 1997 232 235
182. D.E. Brown, M.A. Thrall, S.U. Walkley, D.A. Wenger, T.W. Mitchell, and M.O. Smith Feline Niemann-Pick disease type C Am J Pathol 144 1994 1412 1415
183. D.E. Sleat, J.A. Wiseman, M. El-Banna, S.M. Price, L. Verot, and M.M. Shen Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport Proc Natl Acad Sci USA 101 2004 5886 5891
184. S. Naureckiene, D.E. Sleat, H. Lackland, A. Fensom, M.T. Vanier, and R. Wattiaux Identification of HE1 as the second gene of Niemann-Pick C disease Science 290 2000 2298 2301
185. E.B. Neufeld, M. Wastney, S. Patel, S. Suresh, A.M. Cooney, and N.K. Dwyer The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo J Biol Chem 274 1999 9627 9635
186. M.E. Higgins, J.P. Davies, F.W. Chen, and Y.A. Ioannou Niemann-Pick C1 is a late endosome-resident protein that transiently associates with lysosomes and the trans-golgi network Mol Gen Metab 68 1999 1 13
187. L. Liscum, and S.L. Sturley Intracellular trafficking of Niemann-Pick C proteins 1 and 2, obligate components of subcellular lipid transport Biochim Biophys Acta 1685 2004 22 27
188. E.D. Carstea, J.A. Morris, K.G. Coleman, S.K. Loftus, D. Zhang, and C. Cummings Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis Science 277 1997 228 231
189. H. Watari, E.J. Blanchette-Mackie, N.K. Dwyer, J.M. Glick, S. Patel, and E.B. Neufeld Niemann-Pick C1 protein: obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization Proc Natl Acad Sci USA 96 1999 805 810
190. H. Watari, E.J. Blanchette-Mackie, N.K. Dwyer, M. Watari, E.B. Neufeld, and S. Patel Mutations in the leucine zipper motif and sterol-sensing domain inactivate the Niemann-Pick C1 glycoprotein J Biol Chem 274 1999 21861 21866
191. H. Watari, E.J. Blanchette-Mackie, N.K. Dwyer, M. Watari, C.G. Burd, and S. Patel Determinants of NPC1 expression and action: key promoter regions, posttranscriptional control and the importance of a "cysteine-rich" loop Exp Cell Res 259 2000 247 256
192. J.P. Davies, and Y.A. Ioannou 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 2000 24367 24374
193. Y.A. Ioannou The structure and function of the Niemann-Pick C1 protein Mol Genet Metab 71 2000 175 181
194. W.L. Greer, M.J. Dobson, G.S. Girouard, D.M. Byers, D.C. Riddell, and P.E. Neumann Mutations in NPC1 highlight a conserved NPC1-specific cysteine-rich domain Am J Hum Genet 65 1999 1252 1260
195. N. Friedland, H.L. Liou, P. Lobel, and A.M. Stock Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease Proc Natl Acad Sci USA 100 2003 2512 2517
196. D.C. Ko, J. Binkley, A. Sidow, and M.P. Scott 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 2003 2518 2525
197. K. Chikh, S. Vey, C. Simonot, M.T. Vanier, and G. Millat Niemann-Pick type C disease: importance of N-glycosylation sites for function and cellular location of the NPC2 protein Mol Genet Metab 83 2004 220 230
198. N. Okamura, S. Kiuchi, M. Tamba, T. Kashima, S. Hiramoto, and T. Baba A porcine homolog of the major secretory protein of human epididymis, HE1, specifically binds cholesterol Biochim Biophys Acta 1438 1999 377 387
199. M.T. Vanier, and G. Millat Structure and function of the NPC2 protein Biochim Biophys Acta 1685 2004 14 21
200. C. Scott, and Y.A. Ioannou The NPC1 protein: structure implies function Biochim Biophys Acta 1685 2004 8 13
201. S. Mukherjee, and F.R. Maxfield Lipid and cholesterol trafficking in NPC Biochim Biophys Acta 1685 2004 28 37
202. E. Ikonen, and M. Holtta-Vuori Cellular pathology of Niemann-Pick type C disease Semin Cell Dev Biol 15 2004 445 454
203. C.F. Roff, E. Goldin, M.E. Comly, J. Blanchette-Mackie, A. Cooney, and R.O. Brady Niemann-Pick type-C disease: deficient intracellular transport of exogenously derived cholesterol Am J Med Genet 42 1992 593 598
204. J.C. Cruz, S. Sugii, C. Yu, and T.-Y. Chang Role of Niemann-Pick type C1 protein in intracellular trafficking of low density lipoprotein-derived cholesterol J Biol Chem 275 2000 4013 4021
205. L. Liscum, R.M. Ruggiero, and J.R. Faust The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts J Cell Biol 108 1989 1625 1636
206. J.C. Cruz, and T.-Y. Chang Fate of endogenously synthesized cholesterol in Niemann-Pick type C1 cells J Biol Chem 275 2000 41309 41316
207. M.T. Vanier Lipid changes in Niemann-Pick disease type C brain: personal experience and review of the literature Neurochem Res 24 1999 481 489
208. M.C. Gondre-Lewis, R. McGlynn, and S.U. Walkley Cholesterol accumulation in NPC1-deficient neurons is ganglioside dependent Curr Biol 13 2003 1324 1329
209. R.H. Lachmann Miglustat. Oxford GlycoSciences/Actelion Curr Opin Investig Drugs 4 2003 472 479
210. R.H. Lachmann, D. te Vruchte, E. Lloyd-Evans, G. Reinkensmeier, D.J. Sillence, and L. Fernandez-Guillen Treatment with miglustat reverses the lipid-trafficking defect in Niemann-Pick disease type C Neurobiol Dis 16 2004 654 658
211. Y. Liu, Y.-P. Wu, R. Wada, E.B. Neufeld, K.A. Mullin, and A.C. Howard Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse Hum Mol Genet 9 2000 1087 1092
212. M. Zhang, N.K. Dwyer, D.C. Love, A. Cooney, M. Comly, and E. Neufeld Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease Proc Natl Acad Sci USA 98 2001 4466 4471
213. Y. Sugimoto, H. Ninomiya, Y. Ohsaki, K. Higaki, J.P. Davies, and Y.A. Ioannou Accumulation of cholera toxin and GM1 ganglioside in the early endosome of Niemann-Pick C1-deficient cells Proc Natl Acad Sci USA 98 2001 12391 12396
214. A. Choudhury, D.K. Sharma, D.L. Marks, and R.E. Pagano Elevated endosomal cholesterol levels in Niemann-Pick cells inhibit rab4 and perturb membrane recycling Mol Biol Cell 15 2004 4500 4511
215. A. Choudhury, M. Dominguez, V. Puri, D.K. Sharma, K. Narita, and C.L. Wheatley Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells J Clin Invest 109 2002 1541 1550
216. S.C. Patel, S. Suresh, U. Kumar, C.Y. Hu, A. Cooney, and E.J. Blanchette-Mackie Localization of Niemann-Pick C1 protein in astrocytes: implications for neuronal degeneration in Niemann-Pick type C disease Proc Natl Acad Sci USA 96 1999 1657 1662
217. T. Falk, W.S. Garver, R.P. Erickson, J.M. Wilson, and A.J. Yool Expression of Niemann-Pick type C transcript in rodent cerebellum in vivo and in vitro Brain Res 839 1999 49 57
218. B. Karten, D.E. Vance, R.B. Campenot, and J.E. Vance Cholesterol accumulates in cell bodies, but is decreased in distal axons, of Niemann-Pick C1-deficient neurons J Neurochem 83 2002 1154 1163
219. A. Prasad, W.A. Fischer, R.A. Maue, and L.P. Henderson Regional and developmental expression of the Npc1 mRNA in the mouse brain J Neurochem 75 2000 1250 1257
220. S. Treiber-Held, R. Distl, V. Meske, F. Albert, and T.G. Ohm Spatial and temporal distribution of intracellular free cholesterol in brains of a Niemann-Pick type C mouse model showing hyperphosphorylated tau protein. Implications for Alzheimer's disease J Pathol 200 2003 95 103
221. W.Y. Ong, R.K. Sundaram, E. Huang, S. Ghoshal, U. Kumar, and P.G. Pentchev Neuronal localization and association of Niemann Pick C2 protein (HE1/NPC2) with the postsynaptic density Neuroscience 128 2004 561 570
222. D.C. German, C.L. Liang, T. Song, U. Yazdani, C. Xie, and J.M. Dietschy Neurodegeneration in the Niemann-Pick C mouse: glial involvement Neuroscience 109 2002 437 450
223. M. Baudry, Y. Yao, D. Simmons, J. Liu, and X. Bi Postnatal development of inflammation in a murine model of Niemann-Pick type C disease: immunohistochemical observations of microglia and astroglia Exp Neurol 184 2003 887 903
224. K. Suzuki, C.C. Parker, P.G. Pentchev, D. Katz, B. Ghetti, and A.N. D'Agostino Neurofibrillary tangles in Niemann-Pick disease type C Acta Neuropathol 89 1995 227 238
225. P.A. March, M.A. Thrall, D.E. Brown, T.W. Mitchell, A.C. Lowenthal, and S.U. Walkley GABAergic neuroaxonal dystrophy and other cytopathological alterations in feline Niemann-Pick disease type C Acta Neuropathol (Berl) 94 1997 164 172
226. S.U. Walkley, and K. Suzuki Consequences of NPC1 and NPC2 loss of function in mammalian neurons Biochim Biophys Acta 1685 2004 48 62
227. Y. Higashi, S. Murayama, P.G. Pentchev, and K. Suzuki Cerebellar degeneration in the Niemann-Pick type C mouse Acta Neuropathol 85 1993 175 184
228. A. Yamada, M. Saji, Y. Ukita, Y. Shinoda, M. Taniguchi, and K. Higaki Progressive neuronal loss in the ventral posterior lateral and medial nuclei of thalamus in Niemann-Pick disease type C mouse brain Brain Dev 23 2001 288 297
229. D.C. German, E.M. Quintero, C.L. Liang, B. Ng, S. Punia, and C. Xie Selective neurodegeneration, without neurofibrillary tangles, in a mouse model of Niemann-Pick C disease J Comp Neurol 433 2001 415 425
230. J.R. Sarna, M. Larouche, H. Marzban, R.V. Sillitoe, D.E. Rancourt, and R. Hawkes Patterned Purkinje cell degeneration in mouse models of Niemann-Pick type C disease J Comp Neurol 456 2003 279 291
231. R.P. Erickson, and O. Bernard Studies on neuronal death in the mouse model of Niemann-Pick C disease J Neurosci Res 68 2002 738 744
232. R. Distl, S. Treiber-Held, F. Albert, V. Meske, K. Harzer, and T.G. Ohm Cholesterol storage and tau pathology in Niemann-Pick type C disease in the brain J Pathol 200 2003 104 111
233. M. Iwamoto, I. Morita, M. Fukuda, S. Murota, S. Ando, and Y. Ohno-Iwashita A biotinylated perfringolysin O derivative: a new probe for detection of cell surface cholesterol Biochim Biophys Acta 1327 1997 222 230
234. P.C. Reid, N. Sakashita, S. Sugii, Y. Ohno-Iwashita, Y. Shimada, and W.F. Hickey A novel cholesterol stain reveals early neuronal cholesterol accumulation in the Niemann-Pick type C1 mouse brain J Lipid Res 45 2004 582 591
235. M. Elleder, A. Jirasek, F. Smid, J. Ledvinova, and G.T. Besley Niemann-Pick disease type C. Study on the nature of the cerebral storage process Acta Neuropathol (Berl) 66 1985 325 336
236. A.H. Futerman, and G. van Meer The cell biology of lysosomal storage disorders Nat Rev Mol Cell Biol 5 2004 554 565
237. S.U. Walkley Cellular pathology of lysosomal storage disorders Brain Pathol 8 1998 175 193
238. S. Takikita, T. Fukuda, I. Mohri, T. Yagi, and K. Suzuki Perturbed myelination process of premyelinating oligodendrocyte in Niemann-Pick type C mouse J Neuropathol Exp Neurol 63 2004 660 673
239. H. Weintraub, A. Abramovici, U. Sandbank, P.G. Pentchev, R.O. Brady, and M. Sekine Neurological mutation characterized by dysmyelination in NCTR-Balb/C mouse with lysosomal lipid storage disease J Neurochem 45 1985 665 672
240. H. Weintraub, A. Abramovici, U. Sandbank, A.D. Booth, P.G. Pentchev, and B. Sela Dysmyelination in NCTR-Balb/C mouse mutant with a lysosomal storage disorder. Morphological survey Acta Neuropathol (Berl) 74 1987 374 381
241. C.F. Roff, J.F. Strauss III, E. Goldin, H. Jaffe, M.C. Patterson, and G.C. Agritellis The murine Niemann-Pick Type C lesion affects testosterone production Endocrinology 133 1993 2913 2923
242. L.D. Griffin, W. Gong, L. Verot, and S.H. Mellon Niemann-Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone Nat Med 10 2004 704 711
243. C. Xie, S.D. Turley, and J.M. Dietschy Cholesterol accumulation in tissues of the Niemann-Pick type C mouse is determined by the rate of lipoprotein-cholesterol uptake through the coated-pit pathway in each organ Proc Natl Acad Sci USA 96 1999 11992 11997
244. C. Xie, D.K. Burns, S.D. Turley, and J.M. Dietschy Cholesterol is sequestered in the brains of mice with Niemann-Pick type C disease but turnover is increased J Neuropathol Exp Neurol 59 2000 1106 1117
245. R.D. Shamburek, P.G. Pentchev, L.A. Zech, J. Blanchette-Mackie, E.D. Carstea, and J.M. VandenBroek 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 38 1997 2422 2435
246. L. Liscum Niemann-Pick Type C mutations cause lipid traffic jam Traffic 1 2000 218 225
247. N.K. Dahl, K.L. Reed, M.A. Daunais, J.R. Faust, and L. Liscum Isolation and characterization of Chinese hamster ovary cells defective in the intracellular metabolism of low density lipoprotein-derived cholesterol J Biol Chem 267 1992 4889 4896
248. C. Xie, E.G. Lund, S.D. Turley, D.W. Russell, and J.M. Dietschy Quantitation of two pathways for cholesterol excretion from the brain in normal mice and mice with neurodegeneration J Lipid Res 44 2003 1780 1789
249. J.F. Goodrum, and P.G. Pentchev Cholesterol reutilization during myelination of regenerating PNS axons is impaired in Niemann-Pick disease type C mice J Neurosci Res 49 1997 389 392
250. K.M. Cadigan, D.M. Spillane, and T.-Y. Chang Isolation and characterization of Chinese hamster ovary cell mutants defective in intracellular low density lipoprotein-cholesterol trafficking J Cell Biol 110 1990 295 308
251. B. Karten, D.E. Vance, R.B. Campenot, and J.E. Vance Trafficking of cholesterol from cell bodies to distal axons in Niemann Pick C1-deficient neurons J Biol Chem 278 2003 4168 4175
252. C.A. Paul, A.K. Boegle, and R.A. Maue Before the loss: neuronal dysfunction in Niemann-Pick Type C disease Biochim Biophys Acta 1685 2004 63 76
253. Y. Tashiro, T. Yamazaki, Y. Shimada, Y. Ohno-Iwashita, and K. Okamoto Axon-dominant localization of cell-surface cholesterol in cultured hippocampal neurons and its disappearance in Niemann-Pick type C model cells Eur J Neurosci 20 2004 2015 2021
254. L.P. Henderson, L. Lin, A. Prasad, C.A. Paul, T.Y. Chang, and R.A. Maue Embryonic striatal neurons from Niemann-Pick type C mice exhibit defects in cholesterol metabolism and neurotrophin responsiveness J Biol Chem 275 2000 20179 20187
255. R.B. Campenot, and B.L. MacInnis Retrograde transport of neurotrophins: fact and function J Neurobiol 58 2004 217 229
256. C.L. Howe, and W.C. Mobley Signaling endosome hypothesis: a cellular mechanism for long distance communication J Neurobiol 58 2004 207 216
257. B.L. MacInnis, and R.B. Campenot Retrograde support of neuronal survival without retrograde transport of nerve growth factor Science 295 2002 1536 1539
258. E.M. Ullian, S.K. Sapperstein, K.S. Christopherson, and B.A. Barres Control of synapse number by glia Science 291 2001 657 661
259. K. Nagler, D.H. Mauch, and F.W. Pfrieger Glia-derived signals induce synapse formation in neurones of the rat central nervous system J Physiol 533 2001 665 679
260. B. Karten, H. Hayashi, G.A. Francis, R.B. Campenot, D.E. Vance, and J.E. Vance Generation and function of astroglial lipoproteins from Niemann-Pick type C1-deficient mice Biochem J 2004
261. A.L. Mutka, S. Lusa, M.D. Linder, E. Jokitalo, O. Kopra, and M. Jauhiainen Secretion of sterols and the NPC2 protein from primary astrocytes J Biol Chem 279 2004 48654 48662
262. S. Suresh, Z. Yan, R.C. Patel, Y.C. Patel, and S.C. Patel Cellular cholesterol storage in the Niemann-Pick Disease Type C mouse is associated with increased expression and defective processing of apolipoprotein D J Neurochem 70 1998 242 251
263. G. Franz, M. Reindl, S.C. Patel, R. Beer, I. Unterrichter, and T. Berger Increased expression of apolipoprotein D following experimental traumatic brain injury J Neurochem 73 1999 1615 1625
264. R.A. Nixon Niemann-Pick type C disease and Alzheimer's disease: the APP-endosome connection fattens up Am J Pathol 164 2004 757 761
265. M. Burns, K. Gaynor, V. Olm, M. Mercken, J. LaFrancois, and L. Wang Presenilin redistribution associated with aberrant cholesterol transport enhances beta-amyloid production in vivo J Neurosci 23 2003 5645 5649
266. H. Runz, J. Rietdorf, I. Tomic, M. de Bernard, K. Beyreuther, and R. Pepperkok Inhibition of intracellular cholesterol transport alters presenilin localization and amyloid precursor protein processing in neuronal cells J Neurosci 22 2002 1679 1689
267. I.A. Auer, M.L. Schmidt, V.M.-Y. Lee, B. Curry, K. Suzuki, and R.-W. Shin Paired helical filament tau (PHFtau) in Niemann-Pick type C disease is similar to PHFtau in Alzheimer's disease Acta Neuropathol 90 1995 547 551
268. S. Love, L.R. Bridges, and C.P. Case Neurofibrillary tangles in Niemann-Pick disease type C Brain 118 Pt 1 1995 119 129
269. N. Sawamura, J.S. Gong, W.S. Garver, R.A. Heidenreich, H. Ninomiya, and K. Ohno Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice J Biol Chem 276 2001 10314 10319
270. R. Distl, V. Meske, and T.G. Ohm Tangle-bearing neurons contain more free cholesterol than adjacent tangle-free neurons Acta Neuropathol (Berl) 101 2001 547 554
271. B. Bu, H. Klunemann, K. Suzuki, J. Li, T. Bird, and L.W. Jin Niemann-Pick disease type C yields possible clue for why cerebellar neurons do not form neurofibrillary tangles Neurobiol Dis 11 2002 285 297
272. M. Zhang, J. Li, P. Chakrabarty, B. Bu, and I. Vincent Cyclin-dependent kinase inhibitors attenuate protein hyperphosphorylation, cytoskeletal lesion formation, and motor defects in Niemann-Pick Type C mice Am J Pathol 165 2004 843 853
273. B.I. Giasson, M.S. Forman, M. Higuchi, L.I. Golbe, C.L. Graves, and P.T. Kotzbauer Initiation and synergistic fibrillization of tau and alpha-synuclein Science 300 2003 636 640
274. Y. Saito, K. Suzuki, C.M. Hulette, and S. Murayama Aberrant phosphorylation of alpha-synuclein in human Niemann-Pick type C1 disease J Neuropathol Exp Neurol 63 2004 323 328
275. Y. Saito, K. Suzuki, E. Nanba, T. Yamamoto, K. Ohno, and S. Murayama Niemann-Pick type C disease: accelerated neurofibrillary tangle formation and amyloid beta deposition associated with apolipoprotein E epsilon 4 homozygosity Ann Neurol 52 2002 351 355
276. A.M. Cataldo, J.L. Barnett, C. Pieroni, and R.A. Nixon Increased neuronal endocytosis and protease delivery to early endosomes in sporadic Alzheimer's disease: neuropathologic evidence for a mechanism of increased beta-amyloidogenesis J Neurosci 17 1997 6142 6151
277. L.W. Jin, F.S. Shie, I. Maezawa, I. Vincent, and T. Bird Intracellular accumulation of amyloidogenic fragments of amyloid-beta precursor protein in neurons with Niemann-Pick type C defects is associated with endosomal abnormalities Am J Pathol 164 2004 975 985