Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells

Journal of Biological Chemistry

Volumn 284, Issue 16, 2009, Pages 10855-10867

  Ratovitski, Tamara ^a   Gucek, Marjan ^a   Jiang, Haibing ^a   Chighladze, Ekaterine ^a   Waldron, Elaine ^a   D'Ambola, James ^a   Zhipeng, Hou ^a   Yideng, Liang ^a   Poirier, Michelle A ^a   Hirschhorn, Ricky R ^a   Graham, Rona ^b   Hayden, Michael R ^b   Cole, Robert N ^a   Ross, Christopher A ^a,c,d  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b HOOD COLLEGE   (United States)

^c UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^d CMSC 8 121 ^*  (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CALPAINS; CASPASES; CLEAVAGE PRODUCTS; CLEAVAGE SITES; DELETION ANALYSIS; EPITOPE MAPPING; HEK293 CELLS; HUNTINGTIN; HUNTINGTON DISEASE; MUTANT HUNTINGTIN; N-TERMINAL; NEURONAL CELL; SPECIFIC DETECTION;

AMINES; AMINO ACIDS; ANTIBODIES; CHROMATOGRAPHIC ANALYSIS; MASS SPECTROMETRY; ORGANIC ACIDS; TOXICITY;

SIZE EXCLUSION CHROMATOGRAPHY;

ARGININE; HUNTINGTIN; MUTANT PROTEIN; PROTEIN ANTIBODY; HD PROTEIN, HUMAN; NERVE PROTEIN; NUCLEAR PROTEIN; PEPTIDE FRAGMENT;

AFFINITY CHROMATOGRAPHY; AMINO TERMINAL SEQUENCE; ARTICLE; CELL AGGREGATION; CONTROLLED STUDY; CYTOTOXICITY; EPITOPE MAPPING; GEL PERMEATION CHROMATOGRAPHY; GENE DELETION; HUMAN; HUMAN CELL; HUNTINGTON CHOREA; MASS SPECTROMETRY; NERVE CELL; PRIORITY JOURNAL; PROTEIN CLEAVAGE; PROTEIN DEGRADATION; PROTEIN EXPRESSION; AMINO ACID SEQUENCE; ANIMAL; CELL LINE; CYTOLOGY; GENETICS; METABOLISM; MOLECULAR GENETICS; PATHOLOGY;

AMINO ACID SEQUENCE; ANIMALS; CELL LINE; HUMANS; HUNTINGTON DISEASE; MOLECULAR SEQUENCE DATA; NERVE TISSUE PROTEINS; NEURONS; NUCLEAR PROTEINS; PEPTIDE FRAGMENTS;

EID: 67449094981     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M804813200     Document Type: Article

References (43)

1. The Huntington's Disease Collaborative Research Group (1993) Cell 72, 971-983
2. Ross, C. A., Margolis, R. L., Rosenblatt, A., Ranen, N. G., Becher, M. W., and Aylward, E. (1997) Medicine (Baltimore) 76, 305-338
3. Becher, M. W., Kotzuk, J. A., Sharp, A. H., Davies, S. W., Bates, G. P., Price, D. L., and Ross, C. A. (1998) Neurobiol. Dis. 4, 387-397
4. DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J. P., and Aronin, N. (1997) Science 277, 1990-1993
5. Lunkes, A., Lindenberg, K. S., Ben-Haiem, L., Weber, C., Devys, D., Landwehrmeyer, G. B., Mandel, J. L., and Trottier, Y. (2002) Mol. Cell 10, 259-269
6. Mende-Mueller, L. M., Toneff, T., Hwang, S. R., Chesselet, M. F., and Hook, V. Y. (2001) J. Neurosci. 21, 1830-1837
7. Schilling, G., Klevytska, A., Tebbenkamp, A. T., Juenemann, K., Cooper, J., Gonzales, V., Slunt, H., Poirer, M., Ross, C. A., and Borchelt, D. R. (2007) J. Neuropathol. Exp. Neurol. 66, 313-320
8. Sun, B., Fan, W., Balciunas, A., Cooper, J. K., Bitan, G., Steavenson, S., Denis, P. E., Young, Y., Adler, B., Daugherty, L., Manoukian, R., Elliott, G., Shen, W., Talvenheimo, J., Teplow, D. B., Haniu, M., Haldankar, R., Wypych, J., Ross, C. A., Citron, M., and Richards, W. G. (2002) Neurobiol. Dis. 11, 111-122
9. Kim, M., Lee, H. S., LaForet, G., McIntyre, C., Martin, E. J., Chang, P., Kim, T. W., Williams, M., Reddy, P. H., Tagle, D., Boyce, F. M., Won, L., Heller, A., Aronin, N., and DiFiglia, M. (1999) J. Neurosci. 19, 964-973
10. Lunkes, A., and Mandel, J. L. (1998) Hum. Mol. Genet. 7, 1355-1361
11. Cooper, J. K., Schilling, G., Peters, M. F., Herring, W. J., Sharp, A. H., Kaminsky, Z., Masone, J., Khan, F. A., Delanoy, M., Borchelt, D. R., Dawson, V. L., Dawson, T. M., and Ross, C. A. (1998) Hum. Mol. Genet. 7, 783-790
12. Igarashi, S., Morita, H., Bennett, K. M., Tanaka, Y., Engelender, S., Peters, M. F., Cooper, J. K., Wood, J. D., Sawa, A., and Ross, C. A. (2003) Neuroreport 14, 565-568
13. Zhou, H., Cao, F., Wang, Z., Yu, Z. X., Nguyen, H. P., Evans, J., Li, S. H., and Li, X. J. (2003) J. Cell Biol. 163, 109-118
14. Gafni, J., and Ellerby, L. M. (2002) J. Neurosci. 22, 4842-4849
15. Mangiarini, L., Sathasivam, K., Seller, M., Cozens, B., Harper, A., Hetherington, C., Lawton, M., Trottier, Y., Lehrach, H., Davies, S. W., and Bates, G. P. (1996) Cell 87, 493-506
16. Schilling, G., Becher, M. W., Sharp, A. H., Jinnah, H. A., Duan, K., Kotzuk, J. A., Slunt, H. H., Ratovitski, T., Cooper, J. K., Jenkins, N. A., Copeland, N. G., Price, D. L., Ross, C. A., and Borchelt, D. R. (1999) Hum. Mol. Genet. 8, 397-407
17. Rubinsztein, D. C. (2002) Trends Genet. 18, 202-209
18. Tanaka, Y., Igarashi, S., Nakamura, M., Gafni, J., Torcassi, C., Schilling, G., Crippen, D., Wood, J. D., Sawa, A., Jenkins, N. A., Copeland, N. G., Borchelt, D. R., Ross, C. A., and Ellerby, L. M. (2006) Neurobiol. Dis. 21, 381-391
19. Wellington, C. L., Ellerby, L. M., Hackam, A. S., Margolis, R. L., Trifiro, M. A., Singaraja, R., McCutcheon, K., Salvesen, G. S., Propp, S. S., Bromm, M., Rowland, K. J., Zhang, T., Rasper, D., Roy, S., Thornberry, N., Pinsky, L., Kakizuka, A., Ross, C. A., Nicholson, D. W., Bredesen, D. E., and Hayden, M. R. (1998) J. Biol. Chem. 273, 9158-9167
20. Wellington, C. L., Singaraja, R., Ellerby, L., Savill, J., Roy, S., Leavitt, B., Cattaneo, E., Hackam, A., Sharp, A., Thornberry, N., Nicholson, D. W., Bredesen, D. E., and Hayden, M. R. (2000) J. Biol. Chem. 275, 19831-19838
21. Kim, Y. J., Yi, Y., Sapp, E., Wang, Y., Cuiffo, B., Kegel, K. B., Qin, Z. H., Aronin, N., and DiFiglia, M. (2001) Proc. Natl. Acad. Sci. U. S. A. 98, 12784-12789
22. Wellington, C. L., Ellerby, L. M., Gutekunst, C. A., Rogers, D., Warby, S., Graham, R. K., Loubser, O., van Raamsdonk, J., Singaraja, R., Yang, Y. Z., Gafni, J., Bredesen, D., Hersch, S. M., Leavitt, B. R., Roy, S., Nicholson, D. W., and Hayden, M. R. (2002) J. Neurosci. 22, 7862-7872
23. Hermel, E., Gafni, J., Propp, S. S., Leavitt, B. R., Wellington, C. L., Young, J. E., Hackam, A. S., Logvinova, A. V., Peel, A. L., Chen, S. F., Hook, V., Singaraja, R., Krajewski, S., Goldsmith, P. C., Ellerby, H. M., Hayden, M. R., Bredesen, D. E., and Ellerby, L. M. (2004) Cell Death Differ. 11, 424-438
24. Sawa, A., Nagata, E., Sutcliffe, S., Dulloor, P., Cascio, M. B., Ozeki, Y., Roy, S., Ross, C. A., and Snyder, S. H. (2005) Neurobiol. Dis. 20, 267-274
25. Graham, R. K., Deng, Y., Slow, E. J., Haigh, B., Bissada, N., Lu, G., Pearson, J., Shehadeh, J., Bertram, L., Murphy, Z., Warby, S. C., Doty, C. N., Roy, S., Wellington, C. L., Leavitt, B. R., Raymond, L. A., Nicholson, D. W., and Hayden, M. R. (2006) Cell 125, 1179-1191
26. Gafni, J., Hermel, E., Young, J. E., Wellington, C. L., Hayden, M. R., and Ellerby, L. M. (2004) J. Biol. Chem. 279, 20211-20220
27. Kim, M., Roh, J. K., Yoon, B. W., Kang, L., Kim, Y. J., Aronin, N., and DiFiglia, M. (2003) Exp. Neurol. 183, 109-115
28. Bizat, N., Hermel, J. M., Boyer, F., Jacquard, C., Creminon, C., Ouary, S., Escartin, C., Hantraye, P., Kajewski, S., and Brouillet, E. (2003) J. Neurosci. 23, 5020-5030
29. Goffredo, D., Rigamonti, D., Tartari, M., De Micheli, A., Verderio, C., Matteoli, M., Zuccato, C., and Cattaneo, E. (2002) J. Biol. Chem. 277, 39594-39598
30. Ratovitski, T., Nakamura, M., D'Ambola, J., Chighladze, E., Liang, Y., Wang, W., Graham, R., Hayden, M. R., Borchelt, D. R., Hirschhorn, R. R., and Ross, C. A. (2007) Cell Cycle 6, 2970-2981
31. Warby, S. C., Doty, C. N., Graham, R. K., Carroll, J. B., Yang, Y. Z., Singaraja, R. R., Overall, C. M., and Hayden, M. R. (2008) Hum. Mol. Genet. 17, 2390-2404
32. Slow, E. J., Graham, R. K., Osmand, A. P., Devon, R. S., Lu, G., Deng, Y., Pearson, J., Vaid, K., Bissada, N., Wetzel, R., Leavitt, B. R., and Hayden, M. R. (2005) Proc. Natl. Acad. Sci. U. S. A. 102, 11402-11407
33. Peters, M. F., and Ross, C. A. (2001) J. Biol. Chem. 276, 3188-3194
34. Coombe, D. R., Nakhoul, A. M., Stevenson, S. M., Peroni, S. E., and Sanderson, C. J. (1998) J. Immunol. Methods 215, 145-150
35. Zha, J., Harada, H., Osipov, K., Jockel, J., Waksman, G., and Korsmeyer, S. J. (1997) J. Biol. Chem. 272, 24101-24104
36. Jiang, H., Nucifora, F. C., Jr., Ross, C. A., and DeFranco, D. B. (2003) Hum. Mol. Genet. 12, 1-12
37. Wen, Y., Hu, M. C., Makino, K., Spohn, B., Bartholomeusz, G., Yan, D. H., and Hung, M. C. (2000) Cancer Res. 60, 6841-6845
38. Cuerrier, D., Moldoveanu, T., and Davies, P. L. (2005) J. Biol. Chem. 280, 40632-40641
39. Stabach, P. R., Cianci, C. D., Glantz, S. B., Zhang, Z., and Morrow, J. S. (1997) Biochemistry 36, 57-65
40. Muchowski, P. J., Ning, K., D'Souza-Schorey, C., and Fields, S. (2002) Proc. Natl. Acad. Sci. U. S. A. 99, 727-732
41. Ross, C. A., and Poirier, M. A. (2005) Nat. Rev. Mol. Cell Biol. 6, 891-898
42. Kim, Y. J., Sapp, E., Cuiffo, B. G., Sobin, L., Yoder, J., Kegel, K. B., Qin, Z. H., Detloff, P., Aronin, N., and DiFiglia, M. (2006) Neurobiol. Dis. 22, 346-356
43. Findeis, M. A. (2007) Pharmacol. Ther. 116, 266-286