Mechanisms and consequences of impaired lipid trafficking in Niemann-Pick type C1-deficient mammalian cells

Biochimica et Biophysica Acta - Molecular and Cell Biology of Lipids

Volumn 1791, Issue 7, 2009, Pages 659-670

  Karten, Barbara ^a   Peake, Kyle B ^b   Vance, Jean E ^b  

^a DALHOUSIE UNIVERSITY   (Canada)

^b UNIVERSITY OF ALBERTA   (Canada)

Author keywords

Brain; Cholesterol; Glia; Glycosphingolipid; Liver; Neurodegeneration; Neuron

Indexed keywords

3ALPHA HYDROXY 5ALPHA PREGNAN 20 ONE; CHOLESTEROL; CURCUMIN; CYCLODEXTRIN; FILIPIN; GLYCOPHOSPHOLIPID; HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE INHIBITOR; LIPID BINDING PROTEIN; MEMBRANE PROTEIN; N (2,2,2 TRIFLUOROETHYL) N [4 [2,2,2 TRIFLUORO 1 HYDROXY 1 (TRIFLUOROMETHOXY)ETHYL]PHENYL]BENZENESULFONAMIDE; NIFEDIPINE; PROBUCOL; SQUALESTATIN;

ASTROCYTE; CELL MIGRATION; CELLULAR DISTRIBUTION; CHOLESTEROL METABOLISM; ENDOSOME; GENE MUTATION; GENE REPRESSION; GLIA CELL; HUMAN; HYPERCHOLESTEROLEMIA; LIPID METABOLISM; LIPID STORAGE; LIPID TRANSPORT; LYSOSOME; MAMMAL CELL; MICROGLIA; NERVE CELL; NERVE FIBER DEGENERATION; NERVE FIBER TRANSPORT; NERVE FUNCTION; NEUROLOGIC DISEASE; NIEMANN PICK DISEASE; NONHUMAN; NPC1 GENE; NPC2 GENE; PRIORITY JOURNAL; PROTEIN STRUCTURE; QUALITY OF LIFE; REVIEW; TRANSPORT VESICLE;

ANIMALS; BIOLOGICAL TRANSPORT; CARRIER PROTEINS; HUMANS; LIPID METABOLISM; MEMBRANE GLYCOPROTEINS; MICE; MICE, KNOCKOUT; MUTATION; NIEMANN-PICK DISEASES; PROTEINS; TRANSPORT VESICLES;

MAMMALIA;

EID: 67649275699     PISSN: 13881981     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbalip.2009.01.025     Document Type: Review

References (225)

1. Vanier M.T., and Millat G. Clin. Genet. 64 (2003) 269-281
2. Crocker A.C., and Farber S. Medicine (Baltimore) 37 (1958) 1-95
3. Brady R.O., Kanfer J.N., Mock M.B., and Fredrickson D.S. Proc. Natl. Acad. Sci. U. S. A. 55 (1966) 366-369
4. Pentchev P.G., Boothe A.D., Kruth H.S., Weintroub H., Stivers J., and Brady R.O. J. Biol. Chem. 259 (1984) 5784-5791
5. Loftus S.K., Morris J.A., Carstea E.D., Gu J.Z., Cummings C., Brown A., Ellison J., Ohno K., Rosenfeld M.A., Tagle D.A., Pentchev P.G., and Pavan W.J. Science 277 (1997) 232-235
6. Liscum L., and Faust J.R. J. Biol. Chem. 262 (1987) 17002-17008
7. Liscum L., Ruggiero R.M., and Faust J.R. J. Cell Biol. 108 (1989) 1625-1636
8. Cadigan K.M., M., Spillane D.M., and Chang T.-Y. J. Cell Biol. 110 (1990) 295-308
9. Dahl N.K., Reed K.L., Daunais M.A., Faust J.R., and Liscum L. J. Biol. Chem. 267 (1992) 4889-4896
10. Pentchev P.G., Comly M.E., Kruth H.S., Tokoro T., Butler J., Sokol J., Filling-Katz M., Quirk J.M., Marshall D.C., Patel S., et al. FASEB J. 1 (1987) 40-45
11. Reagan J.W., Hubbert M.L., and Shelness G.S. J. Biol. Chem. 275 (2000) 38104-38110
12. Carstea E.D., Polymeropoulos M.H., Parker C.C., Detera-Wadleigh S.D., O'Neill R.R., Patterson M.C., Goldin E., Xiao H., Straub R.E., Vanier M.T., et al. Proc. Natl. Acad. Sci. U. S. A. 90 (1993) 2002-2004
13. Carstea E.D., Morris J.A., Coleman K.G., Loftus S.K., Zhang D., Cummings C., Gu J., Rosenfeld M.A., Pavan W.J., Krizman D.B., Nagle J., Polymeropoulos M.H., Sturley S.L., Ioannou Y.A., Higgins M.E., Comly M., Cooney A., Brown A., Kaneski C.R., Blanchette-Mackie E.J., Dwyer N.K., Neufeld E.B., Chang T.Y., Liscum L., Strauss III J.F., Ohno K., Zeigler M., Carmi R., Sokol J., Markie D., O'Neill R.R., van Diggelen O.P., Elleder M., Patterson M.C., Brady R.O., Vanier M.T., Pentchev P.G., and Tagle D.A. Science 277 (1997) 228-231
14. Naureckiene S., Sleat D.E., Lackland H., Fensom A., Vanier M.T., Wattiaux R., Jadot M., and Lobel P. Science 290 (2000) 2298-2301
15. Sokol J., Blanchette-Mackie E.J., Kruth H.S., Dwyer N.K., Amende L.M., Butler J.D., Robinson E., Patel S., Brady R.O., Comly M.E., Vanier M.T., and Pentchev P.G. J. Biol. Chem. 263 (1988) 3411-3417
16. Liu B., Li H., Repa J.J., Turley S.D., and Dietschy J.M. J. Lipid Res. 49 (2008) 663-669
17. Sleat D.E., Wiseman J.A., El-Banna M., Price S.M., Verot L., Shen M.M., Tint G.S., Vanier M.T., Walkley S.U., and Lobel P. Proc. Natl. Acad. Sci. U. S. A. 101 (2004) 5886-5891
18. March P.A., Thrall M.A., Brown D.E., Mitchell T.W., Lowenthal A.C., and Walkley S.U. Acta Neuropathol. (Berl) 94 (1997) 164-172
19. Somers K.L., Brown D.E., Fulton R., Schultheiss P.C., Hamar D., Smith M.O., Allison R., Connally H.E., Just C., Mitchell T.W., Wenger D.A., and Thrall M.A. J. Inherit. Metab. Dis. 24 (2001) 427-436
20. Vite C.H., Ding W., Bryan C., O'Donnell P., Cullen K., Aleman D., Haskins M.E., and Van Winkle T. Pediatr. Res. 64 (2008) 544-549
21. Huang X., Suyama K., Buchanan J., Zhu A.J., and Scott M.P. Development 132 (2005) 5115-5124
22. Malathi K., Higaki K., Tinkelenberg A.H., Balderes D.A., Almanzar-Paramio D., Wilcox L.J., Erdeniz N., Redican F., Padamsee M., Liu Y., Khan S., Alcantara F., Carstea E.D., Morris J.A., and Sturley S.L. J. Cell Biol. 164 (2004) 547-556
23. Yang H. Trends Cell Biol. 16 (2006) 427-432
24. Neufeld E.B., Wastney M., Patel S., Suresh S., Cooney A.M., Dwyer N.K., Roff C.F., Ohno K., Morris J.A., Carstea E.D., Incardona J.P., Strauss J.F., Vanier M.T., Patterson M.C., Brady R.O., Pnetchev P.G., and Blanchette-Mackie E.J. J. Biol. Chem. 274 (1999) 9627-9635
25. Patel S.C., Suresh S., Kumar U., Hu C.Y., Cooney A., Blanchette-Mackie E.J., Neufeld E.B., Patel R.C., Brady R.O., Patel Y.C., Pentchev P.G., and Ong W.Y. Proc. Natl. Acad. Sci. U. S. A. 96 (1999) 1657-1662
26. Higgins M.E., Davies J.P., Chen F.W., and Ioannou Y.A. Mol. Gen. Metab. 68 (1999) 1-13
27. Watari H., Blanchette-Mackie E.J., Dwyer N.K., Sun G., Glick J.M., Patel S., Neufeld E.B., Pentchev P.G., and Strauss J.F. Exp. Cell Res. 255 (2000) 56-66
28. Garver W.S., Heidenreich R.A., Erickson R.P., Thomas M.A., and Wilson J.M. J. Lipid Res. 41 (2000) 673-687
29. Frolov A., Srivastava K., Daphna-Iken D., Traub L.M., Schaffer J.E., and Ory D.S. J. Biol. Chem. 276 (2001) 46414-46421
30. Zhang M., Dwyer N.K., Neufeld E.B., Love D.C., Cooney A., Comly M., Patel S., Watari H., Strauss J.F., Pentchev P.G., Hanover J.A., and Blanchette-Mackie E.J. J. Biol. Chem. 276 (2001) 3417-3425
31. Garver W.S., Xie C., Repa J.J., Turley S.D., and Dietschy J.M. J. Lipid Res. 46 (2005) 1745-1754
32. Watari H., Blanchette-Mackie E.J., Dwyer N.K., Watari M., Burd C.G., Patel S., Pentchev P.G., and Strauss III J.F. Exp. Cell Res. 259 (2000) 247-256
33. Garver W.S., Jelinek D., Francis G.A., and Murphy B.D. J. Lipid Res. 49 (2008) 1090-1102
34. Gevry N., Schoonjans K., Guay F., and Murphy B.D. J. Lipid Res. 49 (2008) 1024-1033
35. Okamura N., Kiuchi S., Tamba M., Kashima T., Hiramoto S., Baba T., Dacheux F., Dacheux J.L., Sugita Y., and Jin Y.Z. Biochim. Biophys. Acta 1438 (1999) 377-387
36. Altmann S.W., Davis Jr. H.R., Zhu L.J., Yao X., Hoos L.M., Tetzloff G., Iyer S.P., Maguire M., Golovko A., Zeng M., Wang L., Murgolo N., and Graziano M.P. Science 303 (2004) 1201-1204
37. Nohturfft A., Brown M.S., and Goldstein J.L. Proc. Natl. Acad. Sci. U. S. A. 95 (1998) 12848-12853
38. Horton J.D., Goldstein J.L., and Brown M.S. J. Clin. Invest. 109 (2002) 1125-1131
39. Ko D.C., Gordon M.D., Jin J.Y., and Scott M.P. Mol. Biol. Cell 12 (2001) 601-614
40. Ohgami N., Ko D.C., Thomas M., Scott M.P., Chang C.C., and Chang T.Y. Proc. Natl. Acad. Sci. U. S. A. 101 (2004) 12473-12478
41. Millard E.E., Gale S.E., Dudley N., Zhang J., Schaffer J.E., and Ory D.S. J. Biol. Chem. 280 (2005) 28581-28590
42. Infante R.E., Abi-Mosleh L., Radhakrishnan A., Dale J.D., Brown M.S., and Goldstein J.L. J. Biol. Chem. 283 (2008) 1052-1063
43. Liu R., Lu P., Chu J.W., and Sharom F.J. J. Biol. Chem. 284 (2009) 1840-1852
44. Infante R.E., Radhakrishnan A., Abi-Mosleh L., Kinch L.N., Wang M.L., Grishin N.V., Goldstein J.L., and Brown M.S. J. Biol. Chem. 283 (2008) 1064-1075
45. Cheruku S.R., Xu Z., Dutia R., Lobel P., and Storch J. J. Biol. Chem. 281 (2006) 31594-31604
46. Xu Z., Farver W., Kodukula S., and Storch J. Biochemistry 47 (2008) 11134-11143
47. Ko D.C., Binkley J., Sidow A., and Scott M.P. Proc. Natl. Acad. Sci. U. S. A. 100 (2003) 2518-2525
48. Friedland N., Liou H.L., Lobel P., and Stock A.M. Proc. Natl. Acad. Sci. U. S. A. 100 (2003) 2512-2517
49. Infante R.E., Wang M.L., Radhakrishnan A., Kwon H.J., Brown M.S., and Goldstein J.L. Proc. Natl. Acad. Sci. U. S. A. 105 (2008) 15287-15292
50. Davies J.P., Chen F.W., and Ioannou Y.A. Science 290 (2000) 2295-2298
51. Passeggio J., and Liscum L. J. Biol. Chem. 280 (2005) 10333-10339
52. Pentchev P.G., Comly M.E., Kruth H.S., Vanier M.T., Wenger D.A., Patel S., and Brady R.O. Proc. Natl. Acad. Sci. U. S. A. 82 (1985) 8247-8251
53. Zervas M., Dobrenis K., and Walkley S.U. J. Neuropathol. Exp. Neurol. 60 (2001) 49-64
54. te Vruchte D., Lloyd-Evans E., Veldman R.J., Neville D.C., Dwek R.A., Platt F.M., van Blitterswijk W.J., and Sillence D.J. J. Biol. Chem. 279 (2004) 26167-26175
55. Lloyd-Evans E., Morgan A.J., He X., Smith D.A., Elliot-Smith E., Sillence D.J., Churchill G.C., Schuchman E.H., Galione A., and Platt F.M. Nat. Med. 14 (2008) 1247-1255
56. Kobayashi T., Beuchat M.H., Lindsay M., Frias S., Palmiter R.D., Sakuraba H., Parton R.G., and Gruenberg J. Nat. Cell Biol. 1 (1999) 113-118
57. Mukherjee S., and Maxfield F.R. Biochim. Biophys. Acta 1685 (2004) 28-37
58. Chang T.Y., Chang C.C., Ohgami N., and Yamauchi Y. Annu. Rev. Cell Dev. Biol. 22 (2006) 129-157
59. Reid P.C., Sakashita N., Sugii S., Ohno-Iwashita Y., Shimada Y., Hickey W.F., and Chang T.Y. J. Lipid Res. 45 (2004) 582-591
60. Goldstein J.L., DeBose-Boyd R.A., and Brown M.S. Cell 124 (2006) 35-46
61. Wojtanik K.M., and Liscum L. J. Biol. Chem. 278 (2003) 14850-14856
62. Shamburek R.D., Pentchev P.G., Zech L.A., Blanchette-Mackie J., Carstea E.D., VandenBroek J.M., Cooper P.S., Neufeld E.B., Phair R.D., Brewer Jr. H.B., Brady R.O., and Schwartz C.C. J. Lipid Res. 38 (1997) 2422-2435
63. Xie C., Turley S.D., and Dietschy J.M. Proc. Natl. Acad. Sci. U. S. A. 96 (1999) 11992-11997
64. Cruz J.C., and Chang T.-Y. J. Biol. Chem. 275 (2000) 41309-41316
65. Hao M., and Maxfield F.R. J. Biol. Chem. 275 (2000) 15279-15286
66. Urano Y., Watanabe H., Murphy S.R., Shibuya Y., Geng Y., Peden A.A., Chang C.C., and Chang T.Y. Proc. Natl. Acad. Sci. U. S. A. 105 (2008) 16513-16518
67. Olkkonen V.M., and Levine T.P. Biochem. Cell Biol. 82 (2004) 87-98
68. Jabs S., Quitsch A., Kakela R., Koch B., Tyynela J., Brade H., Glatzel M., Walkley S., Saftig P., Vanier M.T., and Braulke T. J. Neurochem. 106 (2008) 1415-1425
69. Chevallier J., Chamoun Z., Jiang G., Prestwich G., Sakai N., Matile S., Parton R.G., and Gruenberg J. J. Biol. Chem. 283 (2008) 27871-27880
70. Puri V., Jefferson J.R., Singh R.D., Wheatley C.L., Marks D.L., and Pagano R.E. J. Biol. Chem. 278 (2003) 20961-20970
71. Vanier M.T. Neurochem. Res. 24 (1999) 481-489
72. Xie C., Burns D.K., Turley S.D., and Dietschy J.M. J. Neuropathol. Exp. Neurol. 59 (2000) 1106-1117
73. German D.C., Liang C.L., Song T., Yazdani U., Xie C., and Dietschy J.M. Neuroscience 109 (2002) 437-450
74. Treiber-Held S., Distl R., Meske V., Albert F., and Ohm T.G. J. Pathol. 200 (2003) 95-103
75. Yamada A., Saji M., Ukita Y., Shinoda Y., Taniguchi M., Higaki K., Ninomiya H., and Ohno K. Brain Dev. 23 (2001) 288-297
76. Distl R., Treiber-Held S., Albert F., Meske V., Harzer K., and Ohm T.G. J. Pathol. 200 (2003) 104-111
77. Karten B., Vance D.E., Campenot R.B., and Vance J.E. J. Neurochem. 83 (2002) 1154-1163
78. Karten B., Vance D.E., Campenot R.B., and Vance J.E. J. Biol. Chem. 278 (2003) 4168-4175
79. Karten B., Hayashi H., Francis G.A., Campenot R.B., Vance D.E., and Vance J.E. Biochem. J. 387 (2005) 779-788
80. Zhang M., Dwyer N.K., Love D.C., Cooney A., Comly M., Neufeld E., Pentchev P.G., Blanchette-Mackie E.J., and Hanover J.A. Proc. Natl. Acad. Sci. U. S. A. 98 (2001) 4466-4471
81. Coxey R.A., Pentchev P.G., Campbell G., and Blanchette-Mackie E.J. J. Lipid Res. 34 (1993) 1165-1176
82. Choudhury A., Sharma D.K., Marks D.L., and Pagano R.E. Mol. Biol. Cell 15 (2004) 4500-4511
83. Ganley I.G., and Pfeffer S.R. J. Biol. Chem. 281 (2006) 17890-17899
84. Choudhury A., Dominguez M., Puri V., Sharma D.K., Narita K., Wheatley C.L., Marks D.L., and Pagano R.E. J. Clin. Invest. 109 (2002) 1541-1550
85. Walter M., Davies J.P., and Ioannou Y.A. J. Lipid Res. 44 (2003) 243-253
86. Narita K., Choudhury A., Dobrenis K., Sharma D.K., Holicky E.L., Marks D.L., Walkley S.U., and Pagano R.E. FASEB J. 19 (2005) 1558-1560
87. Linder M.D., Uronen R.L., Holtta-Vuori M., van der Sluijs P., Peranen J., and Ikonen E. Mol. Biol. Cell 18 (2007) 47-56
88. Kaptzan T., West S.A., Holicky E.L., Wheatley C.L., Marks D.L., Wang T., Peake K.B., Vance J., Walkley S.U., and Pagano R.E. Am. J. Pathol. 174 (2009) 14-20
89. Valenza M., Rigamonti D., Goffredo D., Zuccato C., Fenu S., Jamot L., Strand A., Tarditi A., Woodman B., Racchi M., Mariotti C., Di Donato S., Corsini A., Bates G., Pruss R., Olson J.M., Sipione S., Tartari M., and Cattaneo E. J. Neurosci. 25 (2005) 9932-9939
90. Dietschy J.M., and Turley S.D. J. Lipid Res. 45 (2004) 1375-1397
91. Pfrieger F.W. BioEssays 25 (2003) 486-490
92. Boyles J.K., Zoellner C.D., Anderson L.J., Kosik L.M., Pitas R.E., Weisgraber K.H., Hui D.Y., Mahley R.W., J. G.-H.P., J. I.M., and Shooter E.M. J. Clin. Invest. 83 (1989) 1015-1031
93. Posse de Chaves E.I., Rusiñol A.E., Vance D.E., Campenot R.B., and Vance J.E. J. Biol. Chem. 272 (1997) 30766-30773
94. Posse de Chaves E.I., Vance D.E., Campenot R.B., Kiss R.S., and Vance J.E. J. Biol. Chem. 275 (2000) 19883-19890
95. Mahley R.W. Science 240 (1988) 622-630
96. Mauch D.H., Nagler K., Schumacher S., Goritz C., Muller E.C., Otto A., and Pfrieger F.W. Science 294 (2001) 1354-1357
97. Goritz C., Mauch D.H., and Pfrieger F.W. Mol. Cell. Neurosci. 29 (2005) 190-201
98. Hayashi H., Campenot R.B., Vance D.E., and Vance J.E. J. Biol. Chem. 279 (2004) 14009-14015
99. Hayashi H.H., Campenot R.B., Vance D.E., and Vance J.E. J. Neurosci. 27 (2007) 1933-1941
100. Tarr P.T., and Edwards P.A. J. Lipid Res. 49 (2008) 169-182
101. Lund E.G., Guileyardo J.M., and Russell D.W. Proc. Natl. Acad. Sci. U. S. A. 96 (1999) 7238-7243
102. Lund E.G., Xie C., Kotti T., Turley S.D., Dietschy J.M., and Russell D.W. J. Biol. Chem. 278 (2003) 22980-22988
103. Xie C., Lund E.G., Turley S.D., Russell D.W., and Dietschy J.M. J. Lipid Res. 44 (2003) 1780-1789
104. Hancock J.F. Nat. Rev., Mol. Cell Biol. 7 (2006) 456-462
105. Pike L.J. J. Lipid Res. 47 (2006) 1597-1598
106. Schmitz G., and Grandl M. Curr. Opin. Clin. Nutr. Metab. Care 11 (2008) 106-112
107. Munro S. Cell 115 (2003) 377-388
108. Henderson L.P., Lin L., Prasad A., Paul C.A., Chang T.Y., and Maue R.A. J. Biol. Chem. 275 (2000) 20179-20187
109. Goodrum J.T., and Pentchev P.G. J. Neurosci. Res. 49 (1997) 389-392
110. Yadid G., Sotnik-Barkai I., Tornatore C., Baker-Cairns B., Harvey-White J., Pentchev P.G., and Goldin E. Brain Res. 799 (1998) 250-256
111. Byun K., Kim J., Cho S.Y., Hutchinson B., Yang S.R., Kang K.S., Cho M., Hwang K., Michikawa M., Jeon Y.W., Paik Y.K., and Lee B. Proteomics 6 (2006) 1230-1236
112. Phillips S.E., Woodruff III E.A., Liang P., Patten M., and Broadie K. J. Neurosci. 28 (2008) 6569-6582
113. Karten B., Campenot R.B., Vance D.E., and Vance J.E. J. Lipid Res. 47 (2006) 504-514
114. Deisz R.A., Meske V., Treiber-Held S., Albert F., and Ohm T.G. Neuroscience 130 (2005) 867-873
115. Wasser C.R., Ertunc M., Liu X., and Kavalali E.T. J. Physiol. 579 (2007) 413-429
116. Yu W., Gong J.S., Ko M., Garver W.S., Yanagisawa K., and Michikawa M. J. Biol. Chem. 280 (2005) 11731-11739
117. Sarna J.R., Larouche M., Marzban H., Sillitoe R.V., Rancourt D.E., and Hawkes R. J. Comp. Neurol. 456 (2003) 279-291
118. Higashi Y., Murayama S., Pentchev P.G., and Suzuki K. Acta Neuropathol. (Berl) 85 (1993) 175-184
119. Li H., Repa J.J., Valasek M.A., Beltroy E.P., Turley S.D., German D.C., and Dietschy J.M. J. Neuropathol. Exp. Neurol. 64 (2005) 323-333
120. German D.C., Quintero E.M., Liang C.L., Ng B., Punia S., Xie C., and Dietschy J.M. J. Comp. Neurol. 433 (2001) 415-425
121. Ong W.Y., Kumar U., Switzer R.C., Sidhu A., Suresh G., Hu C.Y., and Patel S.C. Exp. Brain Res. 141 (2001) 218-231
122. Luan Z., Saito Y., Miyata H., Ohama E., Ninomiya H., and Ohno K. J. Neurol. Sci. 268 (2008) 108-116
123. Wu Y.P., Mizukami H., Matsuda J., Saito Y., Proia R.L., and Suzuki K. Mol. Genet. Metab. 84 (2005) 9-17
124. Agassandian M., Zhou J., Tephly L.A., Ryan A.J., Carter A.B., and Mallampalli R.K. J. Biol. Chem. 280 (2005) 21577-21587
125. Rimkunas V.M., Graham M.J., Crooke R.M., and Liscum L. J. Lipid Res. 50 (2009) 327-333
126. Alvarez A.R., Klein A., Castro J., Cancino G.I., Amigo J., Mosqueira M., Vargas L.M., Yevenes L.F., Bronfman F.C., and Zanlungo S. FASEB J. 22 (2008) 3617-3627
127. Chau B.N., Chen T.T., Wan Y.Y., DeGregori J., and Wang J.Y. Mol. Cell. Biol. 24 (2004) 4438-4447
128. Erickson R.P., and Bernard O. J. Neurosci. Res. 68 (2002) 738-744
129. Arstila A.U., and Trump B.F. Am. J. Pathol. 53 (1968) 687-733
130. Dunn Jr. W.A. J. Cell Biol. 110 (1990) 1935-1945
131. Klionsky D.J., and Emr S.D. Science 290 (2000) 1717-1721
132. Chu C.T. J. Neuropathol. Exp. Neurol. 65 (2006) 423-432
133. Komatsu M., Waguri S., Chiba T., Murata S., Iwata J., Tanida I., Ueno T., Koike M., Uchiyama Y., Kominami E., and Tanaka K. Nature 441 (2006) 880-884
134. Hara T., Nakamura K., Matsui M., Yamamoto A., Nakahara Y., Suzuki-Migishima R., Yokoyama M., Mishima K., Saito I., Okano H., and Mizushima N. Nature 441 (2006) 885-889
135. Kuma A., Hatano M., Matsui M., Yamamoto A., Nakaya H., Yoshimori T., Ohsumi Y., Tokuhisa T., and Mizushima N. Nature 432 (2004) 1032-1036
136. Shimizu S., Kanaseki T., Mizushima N., Mizuta T., Arakawa-Kobayashi S., Thompson C.B., and Tsujimoto Y. Nat. Cell Biol. 6 (2004) 1221-1228
137. Ko D.C., Milenkovic L., Beier S.M., Manuel H., Buchanan J., and Scott M.P. PLoS Genet. 1 (2005) 81-95
138. Pacheco C.D., Kunkel R., and Lieberman A.P. Hum. Mol. Genet. 16 (2007) 1495-1503
139. Liao G., Yao Y., Liu J., Yu Z., Cheung S., Xie A., Liang X., and Bi X. Am. J. Pathol. 171 (2007) 962-975
140. Kabeya Y., Mizushima N., Ueno T., Yamamoto A., Kirisako T., Noda T., Kominami E., Ohsumi Y., and Yoshimori T. EMBO J. 19 (2000) 5720-5728
141. Cheng J., Ohsaki Y., Tauchi-Sato K., Fujita A., and Fujimoto T. Biochem. Biophys. Res. Commun. 351 (2006) 246-252
142. Komatsu M., Waguri S., Koike M., Sou Y.S., Ueno T., Hara T., Mizushima N., Iwata J., Ezaki J., Murata S., Hamazaki J., Nishito Y., Iemura S., Natsume T., Yanagawa T., Uwayama J., Warabi E., Yoshida H., Ishii T., Kobayashi A., Yamamoto M., Yue Z., Uchiyama Y., Kominami E., and Tanaka K. Cell 131 (2007) 1149-1163
143. Suzuki H., Sakiyama T., Harada N., Abe M., and Tadokoro M. Pediatr. Int. 45 (2003) 1-4
144. Elleder M., Jirasek A., Smid F., Ledvinova J., Besley G.T., and Stopekova M. Virchows Arch., A Pathol. Anat. Histopathol. 402 (1984) 307-317
145. Sugimoto Y., Ninomiya H., Ohsaki Y., Higaki K., Davies J.P., Ioannou Y.A., and Ohno K. Proc. Natl. Acad. Sci. U. S. A. 98 (2001) 12391-12396
146. Taniguchi M., Shinoda Y., Ninomiya H., Vanier M.T., and Ohno K. Brain Dev. 23 (2001) 414-421
147. Zervas M., Somers K.L., Thrall M.A., and Walkley S.U. Curr. Biol. 11 (2001) 1283-1287
148. Walkley S.U., and Suzuki K. Biochim. Biophys. Acta 1685 (2004) 48-62
149. Walkley S.U. Philos. Trans. R. Soc. Lond. B Biol. Sci. 358 (2003) 893-904
150. Bu B., Li J., Davies P., and Vincent I. J. Neurosci. 22 (2002) 6515-6525
151. Ohara S., Ukita Y., Ninomiya H., and Ohno K. Exp. Neurol. 187 (2004) 289-298
152. Love S., Bridges L.R., and Case C.P. Brain 118 Pt 1 (1995) 119-129
153. Suzuki K., Parker C.C., Pentchev P.G., Katz D., Ghetti B., D'Agostino A.N., and Carstea E.D. Acta Neuropathol. (Berl) 89 (1995) 227-238
154. Auer I.A., Schmidt M.L., Lee V.M., Curry B., Suzuki K., Shin R.W., Pentchev P.G., Carstea E.D., and Trojanowski J.Q. Acta Neuropathol. (Berl) 90 (1995) 547-551
155. Leroy K., Bretteville A., Schindowski K., Gilissen E., Authelet M., De Decker R., Yilmaz Z., Buee L., and Brion J.P. Am. J. Pathol. 171 (2007) 976-992
156. Fan Q.W., Yu W., Senda T., Yanagisawa K., and Michikawa M. J. Neurochem. 76 (2001) 391-400
157. Meske V., Albert F., Richter D., Schwarze J., and Ohm T.G. Eur. J. Neurosci. 17 (2003) 93-102
158. Sawamura N., Gong J.S., Garver W.S., Heidenreich R.A., Ninomiya H., Ohno K., Yanagisawa K., and Michikawa M. J. Biol. Chem. 276 (2001) 10314-10319
159. Bu B., Klunemann H., Suzuki K., Li J., Bird T., Jin L.W., and Vincent I. Neurobiol. Dis. 11 (2002) 285-297
160. Dodart J.C., Mathis C., Bales K.R., and Paul S.M. Genes Brain Behav. 1 (2002) 142-155
161. Cruz J.C., and Tsai L.H. Curr. Opin. Neurobiol. 14 (2004) 390-394
162. Zhang M., Li J., Chakrabarty P., Bu B., and Vincent I. Am. J. Pathol. 165 (2004) 843-853
163. Altmann S.W., Davis Jr. H.R., Yao X., Laverty M., Compton D.S., Zhu L.J., Crona J.H., Caplen M.A., Hoos L.M., Tetzloff G., Priestley T., Burnett D.A., Strader C.D., and Graziano M.P. Biochim. Biophys. Acta 1580 (2002) 77-93
164. Hallows J.L., Iosif R.E., Biasell R.D., and Vincent I. J. Neurosci. 26 (2006) 2738-2744
165. Patel S.C., Suresh S., Kumar U., Hu C.Y., Cooney A., Blanchette-Mackie E.J., Neufeld E.B., Patel R.C., Brady R.O., Patel Y.C., Pentchev P.G., and Ong W.-Y. Proc. Natl. Acad. Sci. U. S. A. 96 (1999) 1657-1662
166. Hu C.Y., Ong W.Y., and Patel S.C. J. Neurocytol. 29 (2000) 765-773
167. Bae J.S., Furuya S., Ahn S.J., Yi S.J., Hirabayashi Y., and Jin H.K. Neurosci. Lett. 381 (2005) 234-236
168. Baudry M., Yao Y., Simmons D., Liu J., and Bi X. Exp. Neurol. 184 (2003) 887-903
169. Li H., Turley S.D., Liu B., Repa J.J., and Dietschy J.M. J. Lipid Res. 49 (2008) 1816-1828
170. Suzuki M., Sugimoto Y., Ohsaki Y., Ueno M., Kato S., Kitamura Y., Hosokawa H., Davies J.P., Ioannou Y.A., Vanier M.T., Ohno K., and Ninomiya H. J. Neurosci. 27 (2007) 1879-1891
171. Kreutzberg G.W. Trends Neurosci. 19 (1996) 312-318
172. Stence N., Waite M., and Dailey M.E. Glia 33 (2001) 256-266
173. Tanga F.Y., Raghavendra V., and DeLeo J.A. Neurochem. Int. 45 (2004) 397-407
174. Graeber M.B., Streit W.J., and Kreutzberg G.W. J. Neurosci. Res. 21 (1988) 18-24
175. McGlade-McCulloh E., Morrissey A.M., Norona F., and Muller K.J. Proc. Natl. Acad. Sci. U. S. A. 86 (1989) 1093-1097
176. Giulian D., Chen J., Ingeman J.E., George J.K., and Noponen M. J. Neurosci. 9 (1989) 4416-4429
177. Patrizio M., and Levi G. Neurosci. Lett. 178 (1994) 184-189
178. Lobsiger C.S., and Cleveland D.W. Nat. Neurosci. 10 (2007) 1355-1360
179. Colton C.A., and Gilbert D.L. FEBS Lett. 223 (1987) 284-288
180. Chao C.C., Hu S., Molitor T.W., Shaskan E.G., and Peterson P.K. J. Immunol. 149 (1992) 2736-2741
181. Buttini M., Appel K., Sauter A., Gebicke-Haerter P.J., and Boddeke H.W. Neuroscience 71 (1996) 1-16
182. Nakamura Y., Si Q.S., and Kataoka K. Neurosci. Res. 35 (1999) 95-100
183. Lindholm D., Castren E., Kiefer R., Zafra F., and Thoenen H. J. Cell Biol. 117 (1992) 395-400
184. Mizuno T., Sawada M., Marunouchi T., and Suzumura A. Biochem. Biophys. Res. Commun. 205 (1994) 1907-1915
185. Nakajima K., Honda S., Tohyama Y., Imai Y., Kohsaka S., and Kurihara T. J. Neurosci. Res. 65 (2001) 322-331
186. McGeer P.L., Itagaki S., Boyes B.E., and McGeer E.G. Neurology 38 (1988) 1285-1291
187. Kawamata T., Akiyama H., Yamada T., and McGeer P.L. Am. J. Pathol. 140 (1992) 691-707
188. McGeer P.L., Schulzer M., and McGeer E.G. Neurology 47 (1996) 425-432
189. Wada R., Tifft C.J., and Proia R.L. Proc. Natl. Acad. Sci. U. S. A. 97 (2000) 10954-10959
190. Yrjanheikki J., Keinanen R., Pellikka M., Hokfelt T., and Koistinaho J. Proc. Natl. Acad. Sci. U. S. A. 95 (1998) 15769-15774
191. Du Y., Ma Z., Lin S., Dodel R.C., Gao F., Bales K.R., Triarhou L.C., Chernet E., Perry K.W., Nelson D.L., Luecke S., Phebus L.A., Bymaster F.P., and Paul S.M. Proc. Natl. Acad. Sci. U. S. A. 98 (2001) 14669-14674
192. Beers D.R., Henkel J.S., Xiao Q., Zhao W., Wang J., Yen A.A., Siklos L., McKercher S.R., and Appel S.H. Proc. Natl. Acad. Sci. U. S. A. 103 (2006) 16021-16026
193. Repa J.J., Li H., Frank-Cannon T.C., Valasek M.A., Turley S.D., Tansey M.G., and Dietschy J.M. J. Neurosci. 27 (2007) 14470-14480
194. Zhang M., Strnatka D., Donohue C., Hallows J.L., Vincent I., and Erickson R.P. J. Neurosci. Res. 86 (2008) 2848-2856
195. Dixit S.S., Sleat D.E., Stock A.M., and Lobel P. Biochem. J. 408 (2007) 1-5
196. Rutledge J.C. Pediatr. Pathol. 9 (1989) 779-784
197. Beltroy E.P., Richardson J.A., Horton J.D., Turley S.D., and Dietschy J.M. Hepatology 42 (2005) 886-893
198. Reif S., Spirer Z., Messer G., Baratz M., Bembi B., and Bujanover Y. Clin. Pediatr. (Phila) 33 (1994) 628-630
199. Kelly D.A., Portmann B., Mowat A.P., Sherlock S., and Lake B.D. J. Pediatr. 123 (1993) 242-247
200. Osono Y., Woollett L.A., Herz J., and Dietschy J.M. J. Clin. Invest. 95 (1995) 1124-1132
201. Kulinski A., and Vance J.E. J. Biol. Chem. 282 (2007) 1627-1637
202. Wang M.D., Franklin V., Sundaram M., Kiss R.S., Ho K., Gallant M., and Marcel Y.L. J. Biol. Chem. 282 (2007) 22525-22533
203. Choi H.Y., Karten B., Chan T., Vance J.E., Greer W.L., Heidenreich R.A., Garver W.S., and Francis G.A. J. Biol. Chem. 278 (2003)
204. Rimkunas V.M., Graham M.J., Crooke R.M., and Liscum L. Hepatology 47 (2008) 1504-1512
205. Pipalia N.H., Huang A., Ralph H., Rujoi M., and Maxfield F.R. J. Lipid Res. 47 (2006) 284-301
206. Patterson M.C., and Platt F. Biochim. Biophys. Acta 1685 (2004) 77-82
207. Liu Y., Wu Y.-P., Wada R., Neufeld E.B., Mullin K.A., Howard A.C., Pentchev P.G., Vanier M.T., Suzuki K., and Proia R.L. Hum. Mol. Genet. 9 (2000) 1087-1092
208. Patterson M.C., Vecchio D., Prady H., Abel L., and Wraith J.E. Lancet Neurol. 6 (2007) 765-772
209. Erickson R.P., Garver W.S., Camargo F., Hossain G.S., and Heidenreich R.A. J. Inherit. Metab. Dis. 23 (2000) 54-62
210. Patterson M.C., Di Bisceglie A.M., Higgins J.J., Abel R.B., Schiffmann R., Parker C.C., Argoff C.E., Grewal R.P., Yu K., Pentchev P.G., et al. Neurology 43 (1993) 61-64
211. Reid P.C., Lin S., Vanier M.T., Ohno-Iwashita Y., Harwood Jr. H.J., Hickey W.F., Chang C.C., and Chang T.Y. J. Neurosci. Methods 168 (2008) 15-25
212. Hirsch-Reinshagen V., Zhou S., Burgess B.L., Bernier L., McIsaac S.A., Chan J.Y., Tansley G.H., Cohn J.S., Hayden M.R., and Wellington C.L. J. Biol. Chem. 279 (2004) 41197-41207
213. Wahrle S.E., Jiang H., Parsadanian M., Legleiter J., Han X., Fryer J.D., Kowalewski T., and Holtzman D.M. J. Biol. Chem. 279 (2004) 40987-40993
214. Karten B., Campenot R.B., Vance D.E., and Vance J.E. J. Biol. Chem. 281 (2006) 4049-4057
215. Boadu E., Choi H.Y., Lee D.W., Waddington E.I., Chan T., Asztalos B., Vance J.E., Chan A., Castro G., and Francis G.A. J. Biol. Chem. (2006)
216. Frolov A., Zielinski S.E., Crowley J.R., Dudley-Rucker N., Schaffer J.E., and Ory D.S. J. Biol. Chem. 278 (2003) 25517-25525
217. Langmade S.J., Gale S.E., Frolov A., Mohri I., Suzuki K., Mellon S.H., Walkley S.U., Covey D.F., Schaffer J.E., and Ory D.S. Proc. Natl. Acad. Sci. U. S. A. 103 (2006) 13807-13812
218. Bensinger S.J., Bradley M.N., Joseph S.B., Zelcer N., Janssen E.M., Hausner M.A., Shih R., Parks J.S., Edwards P.A., Jamieson B.D., and Tontonoz P. Cell 134 (2008) 97-111
219. Griffin L.D., Gong W., Verot L., and Mellon S.H. Nat. Med. 10 (2004) 704-711
220. Mellon S., Gong W., and Griffin L.D. Endocr. Res. 30 (2004) 727-735
221. S. Zampieri, S.H. Mellon, T.D. Butters, M. Nevyjel, D.F. Covey, B. Bembi, A. Dardis, J. Cell. Mol. Med. (in press).
222. Strimpakos A.S., and Sharma R.A. Antioxid. Redox Signal. 10 (2008) 511-545
223. Anand P., Thomas S.G., Kunnumakkara A.B., Sundaram C., Harikumar K.B., Sung B., Tharakan S.T., Misra K., Priyadarsini I.K., Rajasekharan K.N., and Aggarwal B.B. Biochem. Pharmacol. 76 (2008) 1590-1611
224. Gelsthorpe M.E., Baumann N., Millard E., Gale S.E., Langmade S.J., Schaffer J.E., and Ory D.S. J. Biol. Chem. 283 (2008) 8229-8236
225. Balch W.E., Morimoto R.I., Dillin A., and Kelly J.W. Science 319 (2008) 916-919