Prion protein misfolding

Current Molecular Medicine

Volumn 9, Issue 7, 2009, Pages 826-835

  Kupfer, L ^a   Hinrichs, W ^b   Groschup, M H ^c  

^a UNIVERSITY OF CALGARY   (Canada)

^b UNIVERSITY OF GREIFSWALD   (Germany)

^c FEDERAL RESEARCH INSTITUTE FOR ANIMAL HEALTH   (Germany)

Author keywords

Misfolding; Prion protein; Structural propensities; Transmissible spongiform encephalopathy

Indexed keywords

AMYLOID; POLYPEPTIDE; PRION PROTEIN; PRION PROTEIN[106-126]; UNCLASSIFIED DRUG;

ALPHA HELIX; AMINO TERMINAL SEQUENCE; BETA SHEET; CARBOXY TERMINAL SEQUENCE; CATALYSIS; CENTRAL NERVOUS SYSTEM; CLINICAL FEATURE; CONFORMATIONAL TRANSITION; DEGENERATIVE DISEASE; HUMAN; MOLECULAR MECHANICS; NEUROTOXICITY; NONHUMAN; PATHOGENESIS; PRION DISEASE; PROTEIN ANALYSIS; PROTEIN FOLDING; PROTEIN STABILITY; PROTEIN STRUCTURE; REVIEW;

ANIMALS; HUMANS; MODELS, MOLECULAR; PEPTIDE FRAGMENTS; PRION DISEASES; PROTEIN CONFORMATION; PROTEIN FOLDING; PRPC PROTEINS; PRPSC PROTEINS;

EID: 70350554445     PISSN: 15665240     EISSN: None     Source Type: Journal    
DOI: 10.2174/156652409789105543     Document Type: Review

References (110)

1. Prusiner, S.B. (1982) Science, 216, 136-144.
2. Chesebro, B. (2003) Br. Med. Bull., 66, 1-20.
3. Dormont, D. (2002). FEBS Lett., 529, 17-21.
4. Adjou, K.T., Deslys, J.P., emaimay, R. and Dormont, D. (1997) Trends Microbiol., 5, 27-31.
5. Modler, A.J., Fabian, H., Sokolowski, F., Lutsch, G., Gast, K. and Damaschun, G. (2004) Amyloid, 11, 215-231.
6. Bessen, R.A. and Marsh, R.F. (1992) J. Gen. Virol., 73(Pt 2), 329-334.
7. Gretzschel, A., Buschmann, A., Eiden, M., Ziegler, U., Luhken, G., Erhardt, G. and Groschup, M.H. (2005) J. Vet. Med., 52, 55-63.
8. Schoch, G., Seeger, H., Bogousslavsky, J., Tolnay, M., Janzer, R.C., Aguzzi, A. and Glatzel, M. (2006) PLoS Med., 3, e14.
9. Vorberg, I. and Priola, S.A. (2002) J. Biol. Chem., 277, 36775-36781.
10. DeArmond, S.J., Sanchez, H., Yehiely, F., Qiu, Y., Ninchak-Casey, A., Daggett, V., Camerino, A.P., Cayetano, J., Rogers, M., Groth, D., Torchia, M., Tremblay, P., Scott, M. R., Cohen, F. E. and Prusiner, S. B. (1997) Neuron, 19, 1337-1348.
11. Rudd, P.M., Merry, A.H., Wormald, M.R. and Dwek, R.A. (2002) Curr. Opin. Struct. Biol., 12, 578-586.
12. Ceciliani, F. and Pergami, P. (2001) Curr. Protein Peptide Sci., 2, 191-204.
13. Croes, E.A. (2004) Expert Opin. Pharmacother., 5, 2391-2396.
14. Koster, T., Singh, K., Zimmermann, M. and Gruys, E. (2003) J. Vet. Pharmacol. Ther., 26, 315-326.
15. Rossi, G., Salmona, M., Forloni, G., Bugiani, O. and Tagliavini, F. (2003) Clin. Lab. Med., 23, 187-208.
16. Campbell, S., Dennehy, U. and Telling, G. (2000) Archiv. Virol., 87-94.
17. Tahiri-Alaoui, A., Gill, A.C., Disterer, P. and James, W. (2004) J. Biol. Chem., 279, 31390-31397.
18. Vorberg, I., Groschup, M.H., Pfaff, E. and Priola, S.A. (2003) J. Virol., 77, 2003-2009.
19. Baskakov, I., Disterer, P., Breydo, L., Shaw, M., Gill, A., James, W. and Tahiri-Alaoui, A. (2005) FEBS Lett., 579, 2589-2596.
20. Lawson, V.A., Priola, S.A., Meade-White, K., Lawson, M. and Chesebro, B. (2004) J. Biol. Chem., 279, 13689-13695.
21. Winklhofer, K.F., Heske, J., Heller, U., Reintjes, A., Muranyi, W., Moarefi, I. and Tatzelt, J. (2003) J. Biol. Chem., 278, 14961-14970.
22. Iovino, M., Falconi, M., Petruzzelli, R. and Desideri, A. (2001) J. Biomol. Struct. Dynamics, 19, 237-246.
23. Rudd, P.M., Wormald, M.R., Wing, D.R., Prusiner, S.B. and Dwek, R.A. (2001) Biochemistry, 40, 3759-3766.
24. Choi, C.J., Kanthasamy, A., Anantharam, V. and Kanthasamy, A.G. (2006) Neurotoxicology, 27, 777-787.
25. Gonzalez-Iglesias, R., Pajares, M.A., Ocal, C., Espinosa, J.C., Oesch, B. and Gasset, M. (2002) J. Mol. Biol., 319, 527-540.
26. Hachiya, N.S., Imagawa, M. and Kaneko, K. (2007) Med. Hypotheses, 68, 670-673.
27. Jin, T., Gu, Y., Zanusso, G., Sy, M., Kumar, A., Cohen, M., Gambetti, P. and Singh, N. (2000) J. Biol. Chem., 275, 38699-38704.
28. Riek, R., Hornemann, S., Wider, G., Billeter, M., Glockshuber, R. and Wuthrich, K. (1996) Nature, 382, 180-182.
29. Campana, V., Sarnataro, D. and Zurzolo, C. (2005) Trends Cell Biol., 15, 102-111.
30. Broome, B.M. and Hecht, M.H. (2000) J. Mol. Biol., 296, 961-968.
31. Govaerts, C., Wille, H., Prusiner, S.B. and Cohen, F.E. (2004) Proc. Natl. Acad. Sci. USA, 101, 8342-8347.
32. Chen, S.G., Zou, W., Parchi, P. and Gambetti, P. (2000) Arch. Virol., 209-216.
33. Hayashi, H.K., Yokoyama, T., Takata, M., Iwamaru, Y., Imamura, M., Ushiki, Y.K. and Shinagawa, M. (2005) Biochem. Biophys. Res. Commun., 328, 1024-1027.
34. Borchelt, D.R., Taraboulos, A. and Prusiner, S.B. (1992) J. Biol. Chem., 267, 16188-16199.
35. Silveira, J.R., Raymond, G.J., Hughson, A.G., Race, R.E., Sim, V.L., Hayes, S.F. and Caughey, B. (2005) Nature, 437, 257-261.
36. Walker, L.C., Levine, H., 3rd, Mattson, M.P. and Jucker, M. (2006) Trends Neurosci., 29, 438-443.
37. Meyer-Luehmann, M., Coomaraswamy, J., Bolmont, T., Kaeser, S., Schaefer, C., Kilger, E., Neuenschwander, A., Abramowski, D., Frey, P., Jaton, A.L., Vigouret, J. M., Paganetti, P., Walsh, D.M., Mathews, P.M., Ghiso, J., Staufenbiel, M, Walker, L.C. and Jucker, M. (2006) Science, 313, 1781-1784.
38. Sunde, M., Serpell, L.C., Bartlam, M., Fraser, P.E., Pepys, M.B. and Blake, C.C. (1997) J. Mol. Biol., 273, 729-739.
39. Bucciantini, M., Giannoni, E., Chiti, F., Baroni, F., Formigli, L., Zurdo, J., Taddei, N., Ramponi, G., Dobson, C.M. and Stefani, M. (2002) Nature, 416, 507-511.
40. Calamai, M., Chiti, F. and Dobson, C.M. (2005) Biophys. J., 89, 4201-4210.
41. Slotta, U., Hess, S., Spiess, K., Stromer, T., Serpell, L. and Scheibel, T. (2007) Macromol. Biosci., 7, 183-188.
42. Exler, J.H., Hummerich, D. and Scheibel, T. (2007) Angew Chem. Int. Ed. Engl., 46, 3559-3562.
43. Dobson, C.M. (2003) Nature, 426, 884-890.
44. Fandrich, M., Fletcher, M.A. and Dobson, C.M. (2001) Nature, 410, 165-166.
45. Baskakov, I.V. (2004) J. Biol. Chem., 279, 7671-7677.
46. Kumita, J.R., Poon, S., Caddy, G.L., Hagan, C.L., Dumoulin, M., Yerbury, J.J., Stewart, E.M., Robinson, C.V., Wilson, M.R. and Dobson, C.M. (2007) J. Mol. Biol., 369, 157-167.
47. DebBurman, S.K., Raymond, G.J., Caughey, B. and Lindquist, S. (1997) Proc. Natl. Acad. Sci. USA, 94, 13938-13943.
48. Yin, S., Pham, N., Yu, S., Li, C., Wong, P., Chang, B., Kang, S.C., Biasini, E., Tien, P., Harris, D.A., Sy, M.S. (2007) Proc. Natl. Acad. Sci. USA, 104, 7546-7551.
49. Snow, A.D., Willmer, J. and Kisilevsky, R. (1987) Lab. Invest., 56, 120-123.
50. Snow, A.D., Kisilevsky, R., Willmer, J., Prusiner, S.B. and DeArmond, S.J. (1989) Acta Neuropathol., 77, 337-342.
51. Wong, C., Xiong, L.W., Horiuchi, M., Raymond, L., Wehrly, K., Chesebro, B. and Caughey, B. (2001) EMBO J., 20, 377-386.
52. Kellings, K., Meyer, N., Mirenda, C., Prusiner, S.B. and Riesner, D. (1992) J. Gen. Virol., 73 ( Pt 4), 1025-1029.
53. Kellings, K., Prusiner, S.B. and Riesner, D. (1994) Philos. Trans. R. Soc. Lond., 343, 425-430.
54. Klein, T.R., Kirsch, D., Kaufmann, R. and Riesner, D. (1998) Biol. Chem., 379, 655-666.
55. Wopfner, F., Weidenhofer, G., Schneider, R., von Brunn, A., Gilch, S., Schwarz, T.F., Werner, T. and Schatzl, H.M. (1999) J. Mol. Biol., 289, 1163-1178.
56. Kovacs, G.G., Laszlo, L., Bakos, A., Minarovits, J., Bishop, M.T., Strobel, T., Vajna, B., Mitrova, E. and Majtenyi, K. (2005) Neurology, 65, 1666-1669.
57. Palmer, M.S., Dryden, A.J., Hughes, J.T. and Collinge, J. (1991) Nature, 352, 340-342.
58. Collinge, J. (2001) Ann. Rev. Neurosci., 24, 519-550.
59. Baker, H.E., Poulter, M., Crow, T.J., Frith, C.D., Lofthouse, R. and Ridley, R.M. (1991) Lancet, 337, 1286.
60. Baskakov, I.V., Aagaard, C., Mehlhorn, I., Wille, H., Groth, D., Baldwin, M.A., Prusiner, S.B. and Cohen, F.E. (2000) Biochemistry, 39, 2792-2804.
61. Rezaei, H., Choiset, Y., Eghiaian, F., Treguer, E., Mentre, P., Debey, P., Grosclaude, J. and Haertle, T. (2002) J. Mol. Biol., 322, 799-814.
62. Bossers, A., Belt, P., Raymond, G.J., Caughey, B., de Vries, R. and Smits, M.A. (1997) Proc. Natl. Acad. Sci. USA, 94, 4931-4936.
63. Eiden, M., Buschmann, A., Kupfer, L. and Groschup, M.H. (2006) J. Vet. Med., 53, 251-256.
64. Kupfer, L., Eiden, M., Buschmann, A. and Groschup, M.H. (2007) Biochim. Biophys. Acta, 1772, 704-713.
65. Baskakov, I.V., Legname, G., Baldwin, M.A., Prusiner, S.B. and Cohen, F.E. (2002) J. Biol. Chem., 277, 21140-21148.
66. Riek, R., Wider, G., Billeter, M., Hornemann, S., Glockshuber, R. and Wuthrich, K. (1998) Proc. Natl. Acad. Sci. USA, 95, 11667-11672.
67. Barrow, C.J., Yasuda, A., Kenny, P.T. and Zagorski, M.G. (1992) J. Mol. Biol., 225, 1075-1093.
68. Kuznetsov, I.B. and Rackovsky, S. (2004) Protein Sci., 13, 3230-3244.
69. Hornemann, S., Schorn, C. and Wuthrich, K. (2004) EMBO Rep., 5, 1159-1164.
70. Calzolai, L., Lysek, D.A., Perez, D.R., Guntert, P. and Wuthrich, K. (2005) Proc. Natl. Acad. Sci. USA, 102, 651-655.
71. Hirschberger, T., Stork, M., Schropp, B., Winklhofer, K.F., Tatzelt, J. and Tavan, P. (2006) Biophys. J., 90, 3908-3918.
72. Santini, S. and Derreumaux, P. (2004) Cell Mol. Life Sci., 61, 951-960.
73. Norstrom, E.M. and Mastrianni, J.A. (2006) J. Virol., 80, 8521-8529.
74. Vorberg, I., Chan, K. and Priola, S.A. (2001) J. Virol., 75, 10024-10032.
75. Speare, J.O., Rush, T.S., 3rd, Bloom, M.E. and Caughey, B. (2003) J. Biol. Chem., 278, 12522-12529.
76. Ziegler, J., Sticht, H., Marx, U.C., Muller, W., Rosch, P. and Schwarzinger, S. (2003) J. Biol. Chem., 278, 50175-50181.
77. Watzlawik, J., Skora, L., Frense, D., Griesinger, C., Zweckstetter, M., Schulz-Schaeffer, W.J. and Kramer, M.L. (2006) J. Biol. Chem., 281, 30242-30250.
78. Young, M., Kirshenbaum, K., Dill, K.A. and Highsmith, S. (1999) Protein Sci., 8, 1752-1764.
79. Nguyen, J., Baldwin, M.A., Cohen, F.E. and Prusiner, S.B. (1995) Biochemistry, 34, 4186-4192.
80. Zhang, H., Kaneko, K., Nguyen, J.T., Livshits, T.L., Baldwin, M.A., Cohen, F.E., James, T.L. and Prusiner, S.B. (1995) J. Mol. Biol., 250, 514-526.
81. Li, A., Christensen, H.M., Stewart, L.R., Roth, K.A., Chiesa, R. and Harris, D.A. (2007) EMBO J., 26, 548-558.
82. Prusiner, S.B. (1998) Proc. Natl. Acad. Sci. USA, 95, 13363-13383.
83. Liu, A., Riek, R., Zahn, R., Hornemann, S., Glockshuber, R. and Wuthrich, K. (1999) Biopolymers, 51, 145-152.
84. Knaus, K.J., Morillas, M., Swietnicki, W., Malone, M., Surewicz, W.K. and Yee, V.C. (2001) Nat. Struct. Biol., 8, 770-774.
85. Haire, L.F., Whyte, S.M., Vasisht, N., Gill, A.C., Verma, C., Dodson, E.J., Dodson, G.G. and Bayley, P.M. (2004) J. Mol. Biol., 336, 1175-1183.
86. Mo, H., Moore, R.C., Cohen, F.E., Westaway, D., Prusiner, S.B., Wright, P.E. and Dyson, H.J. (2001) Proc. Natl. Acad. Sci. USA, 98, 2352-2357.
87. Gossert, A.D., Bonjour, S., Lysek, D.A., Fiorito, F. and Wuthrich, K. (2005) Proc. Natl. Acad. Sci. USA, 102, 646-650.
88. Sigurdson, C.J. and Aguzzi, A. (2007) Biochim. Biophys. Acta, 1772, 610-618.
89. 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. USA, 94, 13452-13457.
90. Flechsig, E., Shmerling, D., Hegyi, I., Raeber, A.J., Fischer, M., Cozzio, A., von Mering, C., Aguzzi, A. and Weissmann, C. (2000) Neuron, 27, 399-408.
91. Gasset, M., Baldwin, M.A., Fletterick, R.J. and Prusiner, S.B. (1993) Proc. Natl. Acad. Sci. USA, 90, 1-5.
92. Viles, J.H., Cohen, F.E., Prusiner, S.B., Goodin, D.B., Wright, P.E. and Dyson, H.J. (1999) Proc. Natl. Acad. Sci. USA, 96, 2042-2047.
93. Kuczius, T., Buschmann, A., Zhang, W., Karch, H., Becker, K., Peters, G. and Groschup, M.H. (2004) Biol. Chem., 385, 739-747.
94. Jones, C.E., Abdelraheim, S.R., Brown, D.R. and Viles, J.H. (2004) J. Biol. Chem., 279, 32018-32027.
95. Stanczak, P., Valensin, D., Porciatti, E., Jankowska, E., Grzonka, Z., Molteni, E., Gaggelli, E., Valensin, G. and Kozlowski, H. (2006) Biochemistry, 45, 12227-12239.
96. Chiesa, R., Drisaldi, B., Quaglio, E., Migheli, A., Piccardo, P., Ghetti, B. and Harris, D.A. (2000) Proc. Natl. Acad. Sci. USA, 97, 5574-5579.
97. Brun, A., Gutierrez-Adan, A., Castilla, J., Pintado, B., Diaz-San Segundo, F., Cano, M.J., Alamillo, E., Espinosa, J.C. and Torres, J.M. (2007) J. Gen. Virol., 88, 1842-1849.
98. Eiden, M., Palm, G.J., Hinrichs, W., Matthey, U., Zahn, R. and Groschup, M.H. (2006) J. Gen. Virol., 87, 3753-3761.
99. Shmerling, D., Hegyi, I., Fischer, M., Blattler, T., Brandner, S., Gotz, J., Rulicke, T., Flechsig, E., Cozzio, A., von Mering, C., Hangartner, C., Aguzzi, A., Weissmann, C. (1998) Cell, 93, 203-214.
100. Silverman, G.L., Qin, K., Moore, R.C., Yang, Y., Mastrangelo, P., Tremblay, P., Prusiner, S.B., Cohen, F.E. and Westaway, D. (2000) J. Biol. Chem., 275, 26834-26841.
101. Nishida, N., Tremblay, P., Sugimoto, T., Shigematsu, K., Shirabe, S., Petromilli, C., Erpel, S.P., Nakaoke, R., Atarashi, R., Houtani, T., Torchia, M., Sakaguchi, S., DeArmond, S. J., Prusiner, S. B., Katamine, S. (1999) Lab. Invest., 79, 689-697.
102. Forloni, G., Angeretti, N., Chiesa, R., Monzani, E., Salmona, M., Bugiani, O. and Tagliavini, F. (1993) Nature, 362, 543-546.
103. Ettaiche, M., Pichot, R., Vincent, J.P. and Chabry, J. (2000) J. Biol. Chem., 275, 36487-36490.
104. Brown, D.R., Schmidt, B., and Kretzschmar, H.A. (1996) Nature, 380, 345-347.
105. Heller, J., Kolbert, A.C., Larsen, R., Ernst, M., Bekker, T., Baldwin, M., Prusiner, S.B., Pines, A. and Wemmer, D.E. (1996) Protein Sci., 5, 1655-1661.
106. Gasset, M., Baldwin, M.A., Lloyd, D.H., Gabriel, J.M., Holtzman, D.M., Cohen, F., Fletterick, R. and Prusiner, S.B. (1992) Proc. Natl. Acad. Sci. USA, 89, 10940-10944.
107. Ziegler, J., Viehrig, C., Geimer, S., Rosch, P. and Schwarzinger, S. (2006) FEBS Lett., 580, 2033-2040.
108. Jobling, M.F., Stewart, L.R., White, A.R., McLean, C., Friedhuber, A., Maher, F., Beyreuther, K., Masters, C.L., Barrow, C.J., Collins, S.J., Cappai, R. (1999) J. Neurochem., 73, 1557-1565.
109. Bergstrom, A.L., Chabry, J., Bastholm, L. and Heegaard, P.M. (2007) Biochim. Biophys. Acta, 1774, 1118-1127.
110. Florio, T., Paludi, D., Villa, V., Principe, D.R., Corsaro, A., Millo, E., Damonte, G., D'Arrigo, C., Russo, C., Schettini, G.. and Aceto, A. (2003) J. Neurochem., 85, 62-72.