Nuclear aggregation of polyglutamine-expanded ataxin-3: fragments escape the cytoplasmic quality control

Journal of Biological Chemistry

Volumn 285, Issue 9, 2010, Pages 6532-6537

  Breuer, Peter ^a   Haacke, Annette ^b,c   Evert, Bernd O ^a   Wüllner, Ullrich ^a  

^a UNIVERSITY OF BONN   (Germany)

^b MAX PLANCK INSTITUTE OF BIOCHEMISTRY   (Germany)

^c NOVARTIS PHARMA AG   (Switzerland)

Author keywords

[No Author keywords available]

Indexed keywords

AGGREGATE FORMATION; ATAXIN-3; CELLULAR STRESS RESPONSE; CO-EXPRESSION; COMMON DENOMINATORS; HEAT SHOCK TRANSCRIPTION FACTORS; INCLUSION FORMATION; NEURODEGENERATION; NEURODEGENERATIVE; NUCLEAR EXPORT; NUCLEAR LOCALIZATION SEQUENCES; POLYGLUTAMINE; POLYGLUTAMINE DISEASE; SUBCELLULAR SITES;

CELL CULTURE; CUSTOMER SATISFACTION; INCLUSIONS; TOTAL QUALITY MANAGEMENT; TRANSCRIPTION FACTORS;

QUALITY CONTROL;

ARGININE; ATAXIN 3; HEAT SHOCK TRANSCRIPTION FACTOR 1; LYSINE; POLYGLUTAMINE; ATXN3 PROTEIN, HUMAN; MJD PROTEIN, MOUSE; NERVE PROTEIN; NUCLEAR PROTEIN; PEPTIDE; PEPTIDE FRAGMENT; REPRESSOR PROTEIN; TRANSCRIPTION FACTOR;

AMINO ACID SEQUENCE; ANIMAL CELL; ARTICLE; CELL NUCLEUS; CELL STRESS; CELLULAR DISTRIBUTION; CONTROLLED STUDY; CYTOPLASM; HUMAN; HUMAN CELL; NONHUMAN; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; PROTEIN TRANSPORT; SIGNAL TRANSDUCTION; SPINOCEREBELLAR DEGENERATION; ACTIVE TRANSPORT; ANIMAL; CELL INCLUSION; CELL LINE; DEGENERATIVE DISEASE; HEAT SHOCK RESPONSE; MACHADO JOSEPH DISEASE; METABOLISM; MOUSE; NUCLEAR LOCALIZATION SIGNAL; PROTEIN MULTIMERIZATION; RAT;

ACTIVE TRANSPORT, CELL NUCLEUS; AMINO ACID SEQUENCE; ANIMALS; CELL LINE; CELL NUCLEUS; CYTOPLASM; HEAT-SHOCK RESPONSE; HUMANS; INCLUSION BODIES; MACHADO-JOSEPH DISEASE; MICE; NERVE TISSUE PROTEINS; NEURODEGENERATIVE DISEASES; NUCLEAR LOCALIZATION SIGNALS; NUCLEAR PROTEINS; PEPTIDE FRAGMENTS; PEPTIDES; PROTEIN MULTIMERIZATION; RATS; REPRESSOR PROTEINS; TRANSCRIPTION FACTORS;

EID: 77949879488     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M109.036335     Document Type: Article

References (35)

1. Dobson, C. M. (2004) Semin. Cell Dev. Biol. 15, 3-16
2. Chai, Y., Wu, L., Griffin, J. D., and Paulson, H. L. (2001) J. Biol. Chem. 276, 44889-44897
3. Cummings, C. J., Mancini, M. A., Antalffy, B., DeFranco, D. B., Orr, H. T., and Zoghbi, H. Y. (1998) Nat. Genet. 19, 148-154
4. Hands, S., Sinadinos, C., and Wyttenbach, A. (2008) Biochim. Biophys. Acta 1779, 507-521
5. Sieradzan, K. A., and Mann, D. M. (2001) Neuropathol. Appl. Neurobiol. 27, 1-21
6. Skinner, P. J., Vierra-Green, C. A., Emamian, E., Zoghbi, H. Y., and Orr, H. T. (2002) Neuromol. Med. 1, 33-42
7. Arrasate, M., Mitra, S., Schweitzer, E. S., Segal, M. R., and Finkbeiner, S. (2004) Nature 431, 805-810
8. Saudou, F., Finkbeiner, S., Devys, D., and Greenberg, M. E. (1998) Cell 95, 55-66
9. Sakahira, H., Breuer, P., Hayer-Hartl, M. K., and Hartl, F. U. (2002) Proc. Natl. Acad. Sci. U.S.A. 99, Suppl. 4, 16412-16418
10. Young, J. C., Agashe, V. R., Siegers, K., and Hartl, F. U. (2004) Nat. Rev. Mol. Cell Biol. 5, 781-791
11. Perez, M. K., Paulson, H. L., Pendse, S. J., Saionz, S. J., Bonini, N. M., and Pittman, R. N. (1998) J. Cell Biol. 143, 1457-1470
12. Fujigasaki, H., Uchihara, T., Koyano, S., Iwabuchi, K., Yagishita, S., Makifuchi, T., Nakamura, A., Ishida, K., Toru, S., Hirai, S., Ishikawa, K., Tanabe, T., and Mizusawa, H. (2000) Exp. Neurol. 165, 248-256
13. Bichelmeier, U., Schmidt, T., Hübener, J., Boy, J., Rüttiger, L., Häbig, K., Poths, S., Bonin, M., Knipper, M., Schmidt, W. J., Wilbertz, J., Wolburg, H., Laccone, F., and Riess, O. (2007) J. Neurosci. 27, 7418-7428
14. Ikeda, H., Yamaguchi, M., Sugai, S., Aze, Y., Narumiya, S., and Kakizuka, A. (1996) Nat. Genet. 13, 196-202
15. Haacke, A., Broadley, S. A., Boteva, R., Tzvetkov, N., Hartl, F. U., and Breuer, P. (2006) Hum. Mol. Genet. 15, 555-568
16. Haacke, A., Hartl, F. U., and Breuer, P. (2007) J. Biol. Chem. 282, 18851-18856
17. Reina, C. P., Zhong, X., and Pittman, R. N. (2009) Hum. Mol. Genet. 19, 235-249
18. Mueller, T., Breuer, P., Schmitt, I., Walter, J., Evert, B. O., and Wüllner, U. (2009) Hum. Mol. Genet. 18, 3334-3343
19. Scherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G. P., Davies, S. W., Lehrach, H., and Wanker, E. E. (1997) Cell 90, 549-558
20. Zuo, J., Rungger, D., and Voellmy, R. (1995) Mol. Cell. Biol. 15, 4319-4330
21. Velazquez, J. M., and Lindquist, S. (1984) Cell 36, 655-662
22. Korolchuk, V. I., Mansilla, A., Menzies, F. M., and Rubinsztein, D. C. (2009) Mol. Cell 33, 517-527
23. Klement, I. A., Skinner, P. J., Kaytor, M. D., Yi, H., Hersch, S. M., Clark, H. B., Zoghbi, H. Y., and Orr, H. T. (1998) Cell 95, 41-53
24. Gidalevitz, T., Ben-Zvi, A., Ho, K. H., Brignull, H. R., and Morimoto, R. I. (2006) Science 311, 1471-1474
25. Tatzelt, J., Zuo, J., Voellmy, R., Scott, M., Hartl, U., Prusiner, S. B., and Welch, W. J. (1995) Proc. Natl. Acad. Sci. U.S.A. 92, 2944-2948
26. Zourlidou, A., Gidalevitz, T., Kristiansen, M., Landles, C., Woodman, B., Wells, D. J., Latchman, D. S., de Belleroche, J., Tabrizi, S. J., Morimoto, R. I., and Bates, G. P. (2007) Hum. Mol. Genet. 16, 1078-1090
27. Winklhofer, K. F., Reintjes, A., Hoener, M. C., Voellmy, R., and Tatzelt, J. (2001) J. Biol. Chem. 276, 45160-45167
28. Lepock, J. R., Frey, H. E., Heynen, M. L., Senisterra, G. A., and Warters, R. L. (2001) Cell Stress Chaperones 6, 136-147
29. Michels, A. A., Nguyen, V. T., Konings, A. W., Kampinga, H. H., and Bensaude, O. (1995) Eur. J. Biochem. 234, 382-389
30. Hageman, J., Vos, M. J., van Waarde, M. A., and Kampinga, H. H. (2007) J. Biol. Chem. 282, 34334-34345
31. Chen, M., Singer, L., Scharf, A., and von Mikecz, A. (2008) J. Cell Biol. 180, 697-704
32. Michels, A. A., Kanon, B., Konings, A. W., Ohtsuka, K., Bensaude, O., and Kampinga, H. H. (1997) J. Biol. Chem. 272, 33283-33289
33. Guigas, G., Kalla, C., and Weiss, M. (2007) FEBS Lett. 581, 5094-5098
34. Bader, N., Jung, T., and Grune, T. (2007) Exp. Gerontol. 42, 864-870
35. Bailey, C. K., Andriola, I. F., Kampinga, H. H., and Merry, D. E. (2002) Hum. Mol. Genet. 11, 515-523