Familial mutations and the thermodynamic stability of the recombinant human prion protein

Journal of Biological Chemistry

Volumn 273, Issue 47, 1998, Pages 31048-81052

  Swietnicki, Wieslaw ^a   Petersen, Robert B ^a   Gambetti, Pierluigi ^a   Surewicz, Witold K ^a  

^a CASE WESTERN RESERVE UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

PRION PROTEIN; RECOMBINANT PROTEIN;

ARTICLE; CIRCULAR DICHROISM; CREUTZFELDT JAKOB DISEASE; FAMILY HISTORY; GENE MUTATION; GERSTMANN STRAUSSLER SCHEINKER SYNDROME; INSOMNIA; POINT MUTATION; PRION; PRIORITY JOURNAL; PROTEIN DENATURATION; PROTEIN FOLDING; PROTEIN SECONDARY STRUCTURE; PROTEIN STABILITY; THERMODYNAMICS;

CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE; GUANIDINE; HEAT; HUMANS; PEPTIDE FRAGMENTS; POINT MUTATION; PRION DISEASES; PRIONS; PROTEIN DENATURATION; RECOMBINANT PROTEINS; SPECTROMETRY, FLUORESCENCE; THERMODYNAMICS;

ESCHERICHIA COLI;

EID: 0032553530     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.273.47.31048     Document Type: Article

References (42)

1. Prusiner, S. B., and DeArmond, S. J. (1994) Annu. Rev. Neurosci. 17, 311-339
2. Weissmann, C. (1996) FEBS Lett. 389, 3-11
3. Parchi, P., Gambetti, P., Picardo, P., and Ghetti, B. (1998) in Progress in Pathology IV (Haddock, G. M., ed) pp. 39-77, Churchill Livingstone, Edinburgh, UK
4. Prusiner, S. B. (1997) Science 278, 245-251
5. Chesebro, B. (1997) Nat. Med. 3, 491-497
6. Aguzzi, A., and Weissmann, C. (1997) Nature 389, 795-798
7. Griffith, J. S. (1967) Nature 215, 1043-1044
8. Prusiner, S. B. (1991) Science 252, 1515-1522
9. Stahl, N., Baldwin, M. A., Teplow, D. B., Hood, L., Gibson, B. W., Burlingame, A. L., and Prusiner, S. B. (1993) Biochemistry 32, 1991-2002
10. Meyer, R. K., McKinley, M. P., Bowman, K. A., Braunfeld, M. B., Barry, R. A., and Prusiner, S. B. (1986) Proc. Natl. Acad. Sci. U. S. A. 83, 2310-2314
11. Oesch, B., Westaway, D., Walchli, M., McKinley, M. P., Kent, S. B. H., Aebersold, R., Barry, R. A., Tempst, P., Teplow, D. B., Hood, L. E., Prusiner, S. B., and Weissmann, C. (1985) Cell 40, 735-746
12. Pan, K. M., Baldwin, M., Nguyen, J., Gasset, M., Serban, A., Groth, D., Mehlhorn, I., Huang, Z., Fletterick, R. J., Cohen, F. E., and Prusiner, S. B. (1993) Proc. Natl. Acad. Sci. U. S. A. 90, 10962-10966
13. Caughey, B. W., Dong, A., Bhat, K. S., Ernst, D., Hayes, S. F., and Caughey, W. S. (1991) Biochemistry 30, 7672-7680
14. Safar, J., Roller, P. P., Gajdusek, D. C., and Gibbs, C. J. (1993) Protein Sci. 2, 2206-2216
15. Riek, R., Hornemann, S., Wider, G., Billeter, M., Glockshuber, R., and Wuthrich, K. (1996) Nature 382, 180-182
16. James, T. L., Liu, H., Ulyanov, N. B., Fair-Jones, S., Zhang, H., Donne, D. G., Kaneko, K., Groth, D., Mehlhorn, I., Prusiner, S. B., and Cohen, F. E. (1997) Proc. Natl. Acad. Sci. U. S. A. 94, 10086-10091
17. Riek, R., Hornemann, S., Wider, G., Glockshuber, R., and Wuthrich, K. (1997) FEBS Lett. 413, 282-288
18. Donne, D. G., Viles, J. H., Groth, D., Mehlhorn, I., James, T. L., Cohen, F. E., Prusiner, S. B., Wright, P. E., and Dyson, H. J. (1997) Proc. Natl. Acad. Sci. U. S. A. 94, 13452-13457
19. Horwich, A. L., and Weissman, J. S. (1977) Cell 89, 499-510
20. Kelly, J. W. (1998) Proc. Natl. Acad. Sci. U. S. A. 95, 930-932
21. Huang, Z., Gabriel, J. M., Baldwin, M. A., Fletterick, R. J., Prusiner, S. B., and Cohen, F. E. (1994) Proc. Natl. Acad. Sci. U. S. A. 91, 7139-7143
22. Cohen, F. E., Pan, K.-M., Huang, Z., Baldwin, M., Fletterick, R. J., and Prusiner, S. B. (1994) Science 264, 530-531
23. Swietnicki, W., Petersen, R., Gambetti, P., and Surewicz, W. K. (1997) J. Biol. Chem. 272, 27517-27520
24. Petersen, R. B., Parchi, P., Richardson, S. L., Urig, C. B., and Gambetti, P. (1996) J. Biol. Chem. 271, 12661-12668
25. Santoro, M. M., and Bolen, D. W. (1988) Biochemistry 27, 8063-8068
26. Clarke, J., and Fersht, A. L. (1993) Biochemistry 32, 4322-4329
27. Herrman, L. M., and Bowler, B. E. (1997) Protein Sci. 6, 657-665
28. Robertson, A. D., and Murphy, K. P. (1997) Chem. Rev. 97, 1251-1267
29. Myers, J. K., Pace, N., and Scholtz, J. M. (1995) Protein Sci. 4, 2138-2148
30. Connelly, P., Ghosaini, L., Hu, C.-Q., Kitamura, S., Tanaka, A., and Sturtevant, J. M. (1991) Biochemistry 30, 1887-1891
31. Barrick, D., and Baldwin, R. L. (1993) Biochemistry 32, 3790-3796
32. Zhang, H., Stockel, J., Mehlhorn, I., Groth, D., Baldwin, M. A., Prusiner, S. B., James, T. L., and Cohen, F. E. (1997) Biochemistry 36, 3543-3553
33. Etfink, M. R., and Ghiron, C. A. (1981) Anal. Biochem. 114, 199-227
34. Hornemann, S., and Glockshuber, R. (1996) J. Mol. Biol. 262, 614-619
35. Kelly, J. W. (1997) Structure (Lond.) 5, 595-600
36. Wetzel, R. (1997) Adv. Protein Chem. 50, 183-242
37. Safar, J. (1996) Semin. Virol. 7, 207-214
38. Booth, D. R., Sunde, M., Bellotti, V., Robinson, C. V., Hutchinson, W. L., Fraser, P. E., Hawkins, P. N., Dobson, C. M., Radford, S. E., Blake, C. C. F., and Pepys, M. B. (1997) Nature 385, 787-793
39. Stockel, J., Safar, J., Wallace, A. C., Cohen, F. E., and Prusiner, S. B. (1998) Biochemistry 37, 7185-7193
40. Kaneko, K., Zulianello, L., Scott, M., Cooper, C. M., Wallace, A. C., James, T. L., Cohen, F. E., and Prusiner, S. B. (1997) Proc. Natl. Acad. Sci. U. S. A. 94, 10069-10074
41. DebBurman, S. K., Raymond, G. J., Caughey, B., and Lindquist, S. (1997) Proc. Natl. Acad. Sci. U. S. A. 94, 13938-13943
42. Lehman, S., and Harris, D. A. (1996) J. Biol. Chem. 271, 1633-1637