Endosomal lipid accumulation in NPC1 leads to inhibition of PKC, hypophosphorylation of vimentin and Rab9 entrapment

Biology of the Cell

Volumn 101, Issue 3, 2009, Pages 141-153

  Walter, Marc ^a   Chen, Fannie W ^a   Tamari, Farshad ^a,b   Wang, Rong ^a   Ioannou, Yiannis A ^a  

^a MOUNT SINAI SCHOOL OF MEDICINE   (United States)

^b KEAN UNIVERSITY   (United States)

Author keywords

Intermediate filament; Lysosomal storage disease; Niemann pick type C1; Rab9; Vimentin

Indexed keywords

CHOLESTEROL; FATTY ACID; GANGLIOSIDE; GLYCOSPHINGOLIPID; LIPID; PROTEIN KINASE C; RAB PROTEIN; RAB9 PROTEIN; UNCLASSIFIED DRUG; VIMENTIN; RAB9A PROTEIN, HUMAN; SPHINGOSINE;

ARTICLE; CONTROLLED STUDY; ENDOSOME; ENZYME INHIBITION; HUMAN; HUMAN CELL; INTERMEDIATE FILAMENT; LIPID TRANSPORT; LIPIDOSIS; LYSOSOME; NIEMANN PICK DISEASE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN BINDING; PROTEIN EXPRESSION; PROTEIN INTERACTION; PROTEIN PHOSPHORYLATION; CELL LINE; CYTOLOGY; DRUG EFFECT; FIBROBLAST; GENE EXPRESSION; GENETICS; LIPID METABOLISM; METABOLISM; PATHOLOGY; PHOSPHORYLATION;

CELL LINE; CHOLESTEROL; ENDOSOMES; FIBROBLASTS; GENE EXPRESSION; HUMANS; INTERMEDIATE FILAMENTS; LIPID METABOLISM; NIEMANN-PICK DISEASE, TYPE C; PHOSPHORYLATION; PROTEIN BINDING; PROTEIN KINASE C; RAB GTP-BINDING PROTEINS; SPHINGOSINE; VIMENTIN;

EID: 60049091317     PISSN: 02484900     EISSN: 1768322X     Source Type: Journal    
DOI: 10.1042/BC20070171     Document Type: Article

References (24)

1. Chen, F.W., Gordon, R.E. and Ioannou, Y.A. (2005) NPC1 late endosomes contain elevated levels of non-esterified ('free') fatty acids and an abnormally glycosylated form of the NPC2 protein. Biochem. J. 390, 549-561
2. Colucci-Guyon, E., Gimenez, Y.R.M., Maurice, T., Babinet, C. and Privat, A. (1999) Cerebellar defect and impaired motor coordination in mice lacking vimentin. Glia 25, 33-43
3. Davies, J. P., Coffer, P.D. and Ioannou, Y.A. (1997) Cloning and mapping of human Rab7 and Rab9 cDNA sequences and identification of a Rab9 pseudogene. Genomics 41, 131-134
4. Eckes, B., Colucci-Guyon, E., Smola, H., Nodder, S., Babinet, C., Krieg, T. and Martin, P. (2000) Impaired wound healing in embryonic and adult mice lacking vimentin. J. Cell Sci. 113, 2455-2462
5. Eriksson, J.E., He, T., Trejo-Skalli, A.V., Harmala-Brasken, A.S., Hellman, J., Chou, Y.H. and Goldman, R.D. (2004) Specific in vivo phosphorylation sites determine the assembly dynamics of vimentin intermediate filaments. J. Cell Sci. 117, 919-932
6. Ganley, I.G. and Pfeffer, S.R. (2006) Cholesterol accumulation sequesters Rab9 and disrupts late endosome function in NPC1-deficient cells. J. Biol. Chem. 281, 17890-17899
7. Goldin, E., Roff, C.F, Miller, S.P., Rodriguez-Lafrasse, C., Vanier, M.T., Brady, R.O. and Pentchev, P.G. (1992) Type C Niemann-Pick disease: a murine model of the lysosomal cholesterol lipidosis accumulates sphingosine and sphinganine in liver. Biochim. Biophys. Acta 1127, 303-311
8. Hannun, Y.A. and Bell, R.M. (1989) Regulation of protein kinase C by sphingosine and lysosphingolipids. Clin. Chim. Acta 185, 333-345
9. Helfand, B.T., Chou, Y.H., Shumaker D.K. and Goldman, R.D. (2005) Intermediate filament proteins participate in signal transduction. Trends Cell Biol. 15, 568-570
10. Herrmann, H. and Aebi, U. (2004) Intermediate filaments: molecular structure, assembly mechanism, and integration into functionally distinct intracellular Scaffolds. Annu. Rev. Biochem. 73, 749-789
11. Inagaki, M., Inagaki, N., Takahashi, T. and Takai, Y. (1997) Phosphorylation-dependent control of structures of intermediate filaments: a novel approach using site- and phosphorylation state-specific antibodies. J. Biochem. (Tokyo) 121, 407-414
12. Ioannou, Y.A. (2004) Defects in transmembrane proteins. In Lysosomal Disorders of the Brain (Platt, F.M. and Walkley, S.U., eds), pp. 206-228, Oxford University Press, New York
13. Ioannou, Y.A., Zeidner, K.M., Grace, M.E. and Desnick, R.J. (1998) Human α-galactosidase A: glycosylation site 3 is essential for enzyme solubility. Biochem. J. 332, 789-797
14. Ivaska, J., Vuoriluoto, K., Huovinen, T, Izawa, I., Inagaki, M. and Parker, P.J. (2005) PKCε-mediated phosphorylation of vimentin controls integrin recycling and motility. EMBO J. 24, 3834-3845
15. Klymkowsky, M.W. (1995) Intermediate filaments: new proteins, some answers, more questions. Curr. Opin. Cell Biol. 7, 46-54
16. Meriane, M., Mary, S., Comunale, F., Vignal, E., Fort, P. and Gauthier-Rouviere, C. (2000) Cdc42Hs and Rac1 GTPases induce the collapse of the vimentin intermediate filament network. J. Biol. Chem. 275, 33046-33052
17. Paramio, J.M. and Jorcano, J.L. (2002) Beyond structure: do intermediate filaments modulate cell signalling? BioEssays 24, 836-844
18. Patterson, M.C., Vanier, M.T., Suzuki, M.C., Morris, J.A., Carstea, E., Neufeld, E.B., Blancheffe-Mackie, E.J. and Pentchev, P.G. (2001) Niemann-Pick disease type C: a lipid trafficking disorder. In The Metabolic and Molecular Bases of Inherited Disease (Scriver, C.R. and Sly, W.S., eds), pp. 3611-3633, McGraw-Hill, New York
19. Perlson, E., Hanz, S., Ben-Yaakov, K., Segal-Ruder, Y., Seger, R. and Fainzilber, M. (2005) Vimentin-dependent spatial translocation of an activated MAP kinase in injured nerve. Neuron 45, 715-726
20. Rodriguez-Lafrasse, C., Rousson, R., Valla, S., Antignac, P., Louisot, P. and Vanier, M.T. (1997) Modulation of protein kinase C by endogenous sphingosine: inhibition of phorbol dibutyrate binding in Niemann-Pick C fibroblasts. Biochem. J. 325, 787-791
21. Sarria, A.J., Panini, S.R. and Evans, R.M. (11992) A functional role for vimentin intermediate filaments in the metabolism of lipoprotein-derived cholesterol in human SW-13 cells. J. Biol. Chem. 267, 19455-19463
22. Soellner, P., Quinlan, R.A. and Franke, W.W. (1985) Identification of a distinct soluble subunit of an intermediate filament protein: tetrameric vimentin from living cells. Proc. Natl. Acad. Sci. U.S.A. 82, 7929-7933
23. Styers, M.L., Kowalczyk, A.P. and Faundez, V. (2005) Intermediate filaments and vesicular membrane traffic: the odd couple's first dance? Traffic 6, 359-365
24. Walter, M., Davies, J.P. and Ioannou, Y.A. (2003) Telomerase immortalization upregulates Rab9 expression and restores LDL cholesterol egress from Niemann-Pick C1 late endosomes. J. Lipid Res. 44, 243-253