Polyglutamine expansion in ataxin-3 does not affect protein stability: Implications for misfolding and disease

Journal of Biological Chemistry

Volumn 279, Issue 46, 2004, Pages 47643-47651

  Chow, Michelle K M ^a   Ellisdon, Andrew M ^a   Cabrita, Lisa D ^a   Bottomley, Stephen P ^a  

^a MONASH UNIVERSITY   (Australia)

Author keywords

[No Author keywords available]

Indexed keywords

AGGLOMERATION; AMINO ACIDS; CONFORMATIONS; DISEASES; NEUROLOGY; PATHOLOGY; PH EFFECTS;

NEURONS; NUCLEAR INCLUSIONS; PROTEIN STABILITY;

PROTEINS;

ATAXIN 3; GLUTAMINE; POLYGLUTAMINE; UNCLASSIFIED DRUG;

ARTICLE; CONFORMATION; CONTROLLED STUDY; FIBER; FLUORESCENCE SPECTROSCOPY; HUMAN; HYPOTHESIS; KINETICS; MODEL; PATHOLOGY; PH; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN ANALYSIS; PROTEIN DENATURATION; PROTEIN FOLDING; PROTEIN FUNCTION; PROTEIN STABILITY; PROTEIN STRUCTURE; PROTEIN SYNTHESIS; PROTEIN VARIANT; TEMPERATURE;

CIRCULAR DICHROISM; HUMANS; HYDROGEN-ION CONCENTRATION; NERVE TISSUE PROTEINS; NEURODEGENERATIVE DISEASES; NUCLEAR PROTEINS; PEPTIDES; PROTEIN CONFORMATION; PROTEIN DENATURATION; PROTEIN FOLDING; PROTEIN ISOFORMS; REPRESSOR PROTEINS;

EID: 9144264986     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.M405799200     Document Type: Article

References (56)

1. Margolis, R. L., and Ross, C. A. (2001) Trends Mol. Med. 7, 479-482
2. Zoghbi, H. Y., and Orr, H. T. (2000) Annu. Rev. Neurosci. 23, 217-247
3. Chai, Y., Berke, S. S., Cohen, R. E., and Paulson, H. L. (2004) J. Biol. Chem. 279, 3605-3611
4. Donaldson, K. M., Li, W., Ching, K. A., Batalov, S., Tsai, C. C., and Joazeiro, C. A. (2003) Proc. Natl. Acad. Sci. U. S. A. 100, 8892-8897
5. Doss-Pepe, E. W., Stenroos, E. S., Johnson, W. G., and Madura, K. (2003) Mol. Cell. Biol. 23, 6469-6483
6. Burnett, B., Li, F., and Pittman, R. N. (2003) Hum. Mol. Genet. 12, 3195-3205
7. Kawaguchi, Y., Okamoto, T., Taniwaki, M., Aizawa, M., Inoue, M., Katayama, S., Kawakami, H., Nakamura, S., Nishimura, M., Akiguchi, I., Kimmura, J., Narumiya, S., and Kakizuka, A. (1994) Nat. Genet. 8, 221-228
8. DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J. P., and Aronin, N. (1997) Science 277, 1990-1993
9. Holmberg, M., Duyckaerts, C., Durr, A., Cancel, G., Gourfinkel-An, I., Damier, P., Faucheux, B., Trottier, Y., Hirsch, E. C., Agid, Y., and Brice, A. (1998) Hum. Mol. Genet. 7, 913-918
10. Paulson, H. L., Perez, M. K., Trottier, Y., Trojanowski, J. Q., Subramony, S. H., Das, S. S., Vig, P., Mandel, J. L., Fischbeck, K. H., and Pittman, R. N. (1997) Neuron 19, 333-344
11. Stefani, M., and Dobson, C. M. (2003) J. Mol. Med. 81, 678-699
12. Chow, M. K., Lomas, D. A., and Bottomley, S. P. (2004) Curr. Med. Chem. 11, 491-499
13. Ellisdon, A. M., and Bottomley, S. P. (2004) IUBMB Life 56, 119-123
14. Ierovnik, E. (2002) Eur. J. Biochem. 269, 3362-3371
15. Dobson, C. M. (2004) Sem. Cell Dev. Biol. 15, 3-16
16. Zander, C., Takahashi, J., El Hachimi, K. H., Fujigasaki, H., Albanese, V., Lebre, A. S., Stevanin, G., Duyckaerts, C., and Brice, A. (2001) Hum. Mol. Genet. 10, 2569-2579
17. 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
18. McGowan, D. P., van Roon-Mom, W., Holloway, H., Bates, G. P., Mangiarini, L., Cooper, G. J., Faull, R. L., and Snell, R. G. (2000) Neuroscience 100, 677-680
19. Bevivino, A. E., and Loll, P. J. (2001) Proc. Natl. Acad. Sci. U. S. A. 98, 11955-11960
20. Chen, S., Berthelier, V., Hamilton, J. B., O'Nuallain, B., and Wetzel, R. (2002) Biochemistry 41, 7391-7399
21. Poirier, M. A., Li, H., Macosko, J., Cai, S., Amzel, M., and Ross, C. A. (2002) J. Biol. Chem. 277, 41032-41037
22. Tanaka, M., Morishima, I., Akagi, T., Hashikawa, T., and Nukina, N. (2001) J. Biol. Chem. 276, 45470-45475
23. Chen, S., Berthelier, V., Yang, W., and Wetzel, R. (2001) J. Mol. Biol. 311, 173-182
24. Chow, M. K., Paulson, H. L., and Bottomley, S. P. (2004) J. Mol. Biol. 335, 333-341
25. Shehi, E., Fusi, P., Secundo, F., Pozzuolo, S., Bairati, A., and Tortora, P. (2003) Biochemistry 42, 14626-14632
26. Scherzinger, E., Sittler, A., Schweiger, K., Heiser, V., Lurz, R., Hasenbank, R., Bates, G. P., Lehrach, H., and Wanker, E. E. (1999) Proc. Natl. Acad. Sci. U. S. A. 96, 4604-4609
27. Marchal, S., Shehi, E., Harricane, M. C., Fusi, P., Heitz, F., Tortora, P., and Lange, R. (2003) J. Biol. Chem. 278, 31554-31563
28. Kelly, J. W. (1998) Curr. Opin. Struct. Biol. 8, 101-106
29. Horwich, A. (2002) J. Clin. Invest. 110, 1221-1232
30. Goto, J., Watanabe, M., Ichikawa, Y., Yee, S. B., Ihara, N., Endo, K., Igarashi, S., Takiyama, Y., Gaspar, C., Maciel, P., Tsuji, S., Rouleau, G. A., and Kanazawa, I. (1997) Neurosci. Res. 28, 373-377
31. van Stokkum, I. H., Spoelder, H. J., Bloemendal, M., van Grondelle, R., and Groen, F. C. (1990) Anal. Biochem. 191, 110-118
32. Provencher, S. W., and Glockner, J. (1981) Biochemistry 20, 33-37
33. Lobley, A., and Wallace, B. A. (2001) Biophys. J. 80, 373a
34. Lobley, A., Whitmore, L., and Wallace, B. A. (2002) Bioinformatics 18, 211-212
35. Bennett, M. J., Huey-Tubman, K. E., Herr, A. B., West, A. P., Jr., Ross, S. A., and Bjorkman, P. J. (2002) Proc. Natl. Acad. Sci. U. S. A. 99, 11634-11639
36. Trottier, Y., Lutz, Y., Stevanin, G., Imbert, G., Devys, D., Cancel, G., Saudou, F., Weber, C., David, G., Tora, L., Agid, Y., Brice, A., and Mandel, J. L. (1995) Nature 378, 403-406
37. Warrick, J. M., Paulson, H. L., Gray-Board, G. L., Bui, Q. T., Fischbeck, K. H., Pittman, R. N., and Bonini, N. M. (1998) Cell 93, 939-949
38. Ding, Q., Lewis, J. J., Strum, K. M., Dimayuga, E., Bruce-Keller, A. J., Dunn, J. C., and Keller, J. N. (2002) J. Biol. Chem. 277, 13935-13942
39. Ladurner, A. G., and Fersht, A. R. (1997) J. Mol. BM. 273, 330-337
40. Chow, M. K., Mackay, J. P., Whisstock, J. C., Scanlon, M. J., and Bottomley, S. P. (2004) Biochem. Biophys. Res. Commun. 322, 387-394
41. Masino, L., Musi, V., Menon, R. P., Fusi, P., Kelly, G., Frenkiel, T. A., Trottier, Y., and Pastore, A. (2003) FEBS Lett. 549, 21-25
42. Dobson, C. M. (2001) Philos. Trans. R. Soc. Lond. B Biol. Sci. 356, 133-145
43. Swietnicki, W., Petersen, R. B., Gambetti, P., and Surewicz, W. K. (1998) J. Biol. Chem. 273, 31048-31052
44. Liemann, S., and Glockshuber, R. (1999) Biochemistry 38, 3258-3267
45. Plakoutsi, G., Taddei, N., Stefani, M., and Chiti, F. (2004) J. Biol. Chem. 279, 14111-14119
46. Shaw, K. L., Grimsley, G. R., Yakovlev, G. I., Makarov, A. A., and Pace, C. N. (2001) Protein Sci. 10, 1206-1215
47. Schmittschmitt, J. P., and Scholtz, J. M. (2003) Protein Sci. 12, 2374-2378
48. Perutz, M. (1994) Protein Sci. 3, 1629-1637
49. Thakur, A. K., and Wetzel, R. (2002) Proc. Natl. Acad. Sci. U. S. A. 99, 17014-17019
50. Monti, M., Garolla di Bard, B. L., Calloni, G., Chiti, F., Amoresano, A., Ramponi, G., and Pucci, P. (2004) J. Mol. Biol. 336, 253-262
51. Chen, S., Ferrone, F. A., and Wetzel, R. (2002) Proc. Natl. Acad. Sci. U. S. A. 99, 11884-11889
52. Nozaki, K., Onodera, O., Takano, H., and Tsuji, S. (2001) Neuroreport 12, 3357-3364
53. Schilling, G., Wood, J. D., Duan, K., Slunt, H. H., Gonzales, V., Yamada, M., Cooper, J. K., Margolis, R. L., Jenkins, N. A., Copeland, N. G., Takahashi, H., Tsuji, S., Price, D. L., Borchelt, D. R., and Ross, C. A. (1999) Neuron 24, 275-286
54. 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
55. Ellerby, L. M., Hackam, A. S., Propp, S. S., Ellerby, H. M., Rabizadeh, S., Cashman, N. R., Trifiro, M. A., Pinsky, L., Wellington, C. L., Salvesen, G. S., Hayden, M. R., and Bredesen, D. E. (1999) J. Neurochem. 72, 185-195
56. 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