Advertisement

Biochemistry (Moscow)

, Volume 72, Issue 13, pp 1519–1536 | Cite as

Prions

  • I. S. Shkundina
  • M. D. Ter-AvanesyanEmail author
Review

Abstract

Prions were originally defined as infectious agents of protein nature, which caused neurodegenerative diseases in animals and humans. The prion concept implies that the infectious agent is a protein in special conformation that can be transmitted to the normal molecules of the same protein through protein-protein interactions. Until the 1990s, the prion phenomenon was associated with the single protein named PrP. Discovery of prions in lower eukaryotes, the yeast Saccharomyces cerevisiae and fungus Podospora anserina, suggests that prions have wider significance. Prions of lower eukaryotes are not related to diseases; their propagation caused by aggregation of prion-like proteins underlies the inheritance of phenotypic traits and most likely has adaptive significance. This review covers prions of mammals and lower eukaryotes, mechanisms of their appearance de novo and maintenance, structure of prion particles, and prospects for the treatment of prion diseases. Recent data concerning the search for new prion-like proteins is included. The paper focuses on the [PSI +] prion of S. cerevisiae, since at present it is the most investigated one. The biological significance of prions is discussed.

Key words

prion PrP neurodegenerative disease non-chromosomal inheritance conformational rearrangement 

Abbreviations

aa

amino acid

GuHCl

guanidine hydrochloride

GFP

green fluorescent protein

ORF

open reading frame

PrD

prion-forming domain

prion

proteinaceous infectious particle

PrP

prion protein

PrPC

normal form of prion protein

PrPSc

infectious form of prion protein

PrPres

in vitro created infectious form of prion protein

Prnp

gene encoding PrP

SDS

sodium dodecyl sulfate

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Chandler, R. L. (1961) Lancet, 1, 1378–1379.PubMedGoogle Scholar
  2. 2.
    Gajdusek, D. C., Gibbs, C. J., and Alpers, M. (1966) Nature, 209, 794–796.PubMedGoogle Scholar
  3. 3.
    Alper, T., Cramp, W. A., Haig, D. A., and Clarke, M. C. (1967) Nature, 209, 764–766.Google Scholar
  4. 4.
    Griffith, J. S. (1967) Nature, 215, 1043–1044.PubMedGoogle Scholar
  5. 5.
    Prusiner, S. B., Garfin, D. E., Cochran, S. P., Baringer, J. R., Hadlow, W. J., Eklund, C. M., and Race, R. E. (1978) Trans. Am. Neurol. Assoc., 103, 62–64.PubMedGoogle Scholar
  6. 6.
    Chesebro, B., Race, R., Wehrly, K., Nishio, J., Bloom, M., Lechner, D., Bergstrom, S., Robbins, K., Mayer, L., Keith, J. M., et al. (1985) Nature, 315, 331–333.PubMedGoogle Scholar
  7. 7.
    Oesch, B., Westaway, D., Walchli, M., McKinley, M. P., Kent, S. B., Aebersold, R., Barry, R. A., Tempst, P., Teplow, D. B., Hood, L. E., et al. (1985) Cell, 40, 735–746.PubMedGoogle Scholar
  8. 8.
    Gabriel, J. M., Oesch, B., Kretzschmar, H., Scott, M., and Prusiner, S. B. (1992) Proc. Natl. Acad. Sci. USA, 89, 9097–9101.PubMedGoogle Scholar
  9. 9.
    Rivera-Milla, E., Stuermer, C. A., and Malaga-Trillo, E. (2003) Trends Genet., 19, 72–75.PubMedGoogle Scholar
  10. 10.
    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.PubMedGoogle Scholar
  11. 11.
    Riek, R., Hornemann, S., Wider, G., Billeter, M., Glockshuber, R., and Wuthrich, K. (1996) Nature, 382, 180–182.PubMedGoogle Scholar
  12. 12.
    Pan, K. M., Baldwin, M., Nguyen, J., Gasset, M., Serban, A., Groth, D., Mehlhorn, I., Huang, Z., Fletterick, R. J., Cohen, F. E., et al. (1993) Proc. Natl. Acad. Sci. USA, 90, 10962–10966.PubMedGoogle Scholar
  13. 13.
    McKinley, M. P., Bolton, D. C., and Prusiner, S. B. (1983) Cell, 35, 57–62.PubMedGoogle Scholar
  14. 14.
    Prusiner, S. B. (1998) Proc. Natl. Acad. Sci. USA, 95, 13363–13383.PubMedGoogle Scholar
  15. 15.
    Bueler, H., Fischer, M., Lang, Y., Bluethmann, H., Lipp, H. P., DeArmond, S. J., Prusiner, S. B., Aguet, M., and Weissmann, C. (1992) Nature, 356, 577–582.PubMedGoogle Scholar
  16. 16.
    Brandner, S., Isenmann, S., Raeber, A., Fischer, M., Sailer, A., Kobayashi, Y., Marino, S., Weissmann, C., and Aguzzi, A. (1996) Nature, 379, 339–343.PubMedGoogle Scholar
  17. 17.
    Blattler, T., Brandner, S., Raeber, A. J., Klein, M. A., Voigtlander, T., Weissmann, C., and Aguzzi, A. (1997) Nature, 389, 69–73.PubMedGoogle Scholar
  18. 18.
    Glatzel, M., and Aguzzi, A. (2000) J. Gen. Virol., 81, 2813–2821.PubMedGoogle Scholar
  19. 19.
    Legname, G., Baskakov, I. V., Nguyen, H. O., Riesner, D., Cohen, F. E., DeArmond, S. J., and Prusiner, S. B. (2004) Science, 305, 673–676.PubMedGoogle Scholar
  20. 20.
    Saborio, G. P., Permanne, B., and Soto, C. (2001) Nature, 411, 810–813.PubMedGoogle Scholar
  21. 21.
    Castilla, J., Saa, P., Hetz, C., and Soto, C. (2005) Cell, 121, 195–206.PubMedGoogle Scholar
  22. 22.
    Pattison, I. H. (1965) in Slow, Latent and Temperate Virus Infections, NINDB Monograph 2 (Gajdusek, D. C., Gibbs, G. J., and Alpers, M. P., eds.) US Government Printing, Washington DC, pp. 249–257.Google Scholar
  23. 23.
    Prusiner, S. B. (1982) Science, 216, 136–144.PubMedGoogle Scholar
  24. 24.
    Clarke, A. R., Jackson, G. S., and Collinge, J. (2001) Philos. Trans. R. Soc. Lond. B Biol. Sci., 356, 185–195.PubMedGoogle Scholar
  25. 25.
    Prusiner, S. B., Scott, M., Foster, D., Pan, K. M., Groth, D., Mirenda, C., Torchia, M., Yang, S. L., Serban, D., Carlson, G. A., et al. (1990) Cell, 63, 673–686.PubMedGoogle Scholar
  26. 26.
    Collinge, J., Palmer, M. S., Sidle, K. C., Gowland, I., Medori, R., Ironside, J., and Lantos, P. (1995) Lancet, 346, 569–570.PubMedGoogle Scholar
  27. 27.
    Bruce, M., Chree, A., McConnell, I., Foster, J., Pearson, G., and Fraser, H. (1994) Philos. Trans. R. Soc. Lond. B Biol. Sci., 343, 405–411.PubMedGoogle Scholar
  28. 28.
    Hill, A. F., Desbruslais, M., Joiner, S., Sidle, K. C., Gowland, I., Collinge, J., Doey, L. J., and Lantos, P. (1997) Nature, 389, 448–450, 526.PubMedGoogle Scholar
  29. 29.
    Bessen, R. A., and Marsh, R. F. (1994) J. Virol., 68, 7859–7868.PubMedGoogle Scholar
  30. 30.
    Bessen, R. A., Kocisko, D. A., Raymond, G. J., Nandan, S., Lansbury, P. T., and Caughey, B. (1995) Nature, 375, 698–700.PubMedGoogle Scholar
  31. 31.
    Caughey, B., Raymond, G. J., and Bessen, R. A. (1998) J. Biol. Chem., 273, 32230–32235.PubMedGoogle Scholar
  32. 32.
    Collinge, J., Sidle, K. C., Meads, J., Ironside, J., and Hill, A. F. (1996) Nature, 383, 685–690.PubMedGoogle Scholar
  33. 33.
    Parchi, P., Castellani, R., Capellari, S., Ghetti, B., Young, K., Chen, S. G., Farlow, M., Dickson, D. W., Sima, A. A., Trojanowski, J. Q., Petersen, R. B., and Gambetti, P. (1996) Ann. Neurol., 39, 767–778.PubMedGoogle Scholar
  34. 34.
    Telling, G. C., Parchi, P., DeArmond, S. J., Cortelli, P., Montagna, P., Gabizon, R., Mastrianni, J., Lugaresi, E., Gambetti, P., and Prusiner, S. B. (1996) Science, 274, 2079–2082.PubMedGoogle Scholar
  35. 35.
    Prusiner, S. B. (1991) Science, 252, 1515–1522.PubMedGoogle Scholar
  36. 36.
    Bellinger-Kawahara, C. G., Kempner, E., Groth, D., Gabizon, R., and Prusiner, S. B. (1988) Virology, 164, 537–541.PubMedGoogle Scholar
  37. 37.
    Jarrett, J. T., and Lansbury, P. T., Jr. (1993) Cell, 73, 1055–1058.PubMedGoogle Scholar
  38. 38.
    Caughey, B., Kocisko, D. A., Raymond, G. J., and Lansbury, P. T., Jr. (1995) Chem. Biol., 2, 807–817.PubMedGoogle Scholar
  39. 39.
    Serio, T. R., Cashikar, A. G., Kowal, A. S., Sawicki, G. J., Moslehi, J. J., Serpell, L., Arnsdorf, M. F., and Lindquist, S. L. (2000) Science, 289, 1317–1321.PubMedGoogle Scholar
  40. 40.
    Wickner, R. B. (1994) Science, 264, 566–569.PubMedGoogle Scholar
  41. 41.
    True, H. L., and Lindquist, S. L. (2000) Nature, 407, 477–483.PubMedGoogle Scholar
  42. 42.
    Coustou, V., Deleu, C., Saupe, S., and Begueret, J. (1997) Proc. Natl. Acad. Sci. USA, 94, 9773–9778.PubMedGoogle Scholar
  43. 43.
    Derkatch, I. L., Bradley, M. E., Hong, J. Y., and Liebman, S. W. (2001) Cell, 106, 171–182.PubMedGoogle Scholar
  44. 44.
    Derkatch, I. L., Bradley, M. E., Zhou, P., Chernoff, Y. O., and Liebman, S. W. (1997) Genetics, 147, 507–519.PubMedGoogle Scholar
  45. 45.
    Volkov, K. V., Aksenova, A. Y., Soom, M. J., Osipov, K. V., Svitin, A. V., Kurischko, C., Shkundina, I. S., Ter-Avanesyan, M. D., Inge-Vechtomov, S. G., and Mironova, L. N. (2002) Genetics, 160, 25–36.PubMedGoogle Scholar
  46. 46.
    Brown, J. C., and Lindquist, S. L. (2005) in Abstracts of Prion Biology (Joint Cold Spring Harbor Laboratory/Wellcome Trust Conference).Google Scholar
  47. 47.
    Beauregard, P. B., Guerin, R., Senechal, P., Collin, P., and Rokeach, L. A. (2005) in Abstracts of Prion Biology (Joint Cold Spring Harbor Laboratory/Wellcome Trust Conference).Google Scholar
  48. 48.
    Collin, P., Beauregard, P. B., Elagoz, A., and Rokeach, L. A. (2004) J. Cell Sci., 117, 907–918.PubMedGoogle Scholar
  49. 49.
    Lacroute, F. (1971) J. Bacteriol., 106, 519–522.PubMedGoogle Scholar
  50. 50.
    Tuite, M. F., Mundy, C. R., and Cox, B. S. (1981) Genetics, 98, 691–711.PubMedGoogle Scholar
  51. 51.
    Aigle, M., and Lacroute, F. (1975) Mol. Gen. Genet., 136, 327–335.PubMedGoogle Scholar
  52. 52.
    Cooper, T. G. (1982) Basic Life Sci., 19, 143–161.PubMedGoogle Scholar
  53. 53.
    Magasanik, B. (1992) in The Molecular and Cellular Biology of the Yeast Saccharomyces, 2nd Edn. (Jones, E. W., Pringle, J. R., and Broach, J. R., eds.) Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY, pp. 283–314.Google Scholar
  54. 54.
    Chisholm, V. T., Lea, H. Z., Rai, R., and Cooper, T. G. (1987) J. Bacteriol., 169, 1684–1690.PubMedGoogle Scholar
  55. 55.
    Cooper, T. G., Ferguson, D., Rai, R., and Bysani, N. (1990) J. Bacteriol., 172, 1014–1018.PubMedGoogle Scholar
  56. 56.
    Courchesne, W. E., and Magasanik, B. (1988) J. Bacteriol., 170, 708–713.PubMedGoogle Scholar
  57. 57.
    Turoscy, V., and Cooper, T. G. (1987) J. Bacteriol., 169, 2598–2600.PubMedGoogle Scholar
  58. 58.
    Masison, D. C., and Wickner, R. B. (1995) Science, 270, 93–95.PubMedGoogle Scholar
  59. 59.
    Edskes, H. K., Gray, V. T., and Wickner, R. B. (1999) Proc. Natl. Acad. Sci. USA, 96, 1498–1503.PubMedGoogle Scholar
  60. 60.
    Komar, A. A., Lesnik, T., Cullin, C., Merrick, W. C., Trachsel, H., and Altmann, M. (2003) EMBO J., 22, 1199–1209.PubMedGoogle Scholar
  61. 61.
    Coschigano, P. W., and Magasanik, B. (1991) Mol. Cell Biol., 11, 822–832.PubMedGoogle Scholar
  62. 62.
    Maddelein, M. L., and Wickner, R. B. (1999) Mol. Cell Biol., 19, 4516–4524.PubMedGoogle Scholar
  63. 63.
    Brachmann, A., Baxa, U., and Wickner, R. B. (2005) EMBO J., 24, 3082–3092.PubMedGoogle Scholar
  64. 64.
    Saupe, S. J. (2000) Microbiol. Mol. Biol. Rev., 64, 489–502.PubMedGoogle Scholar
  65. 65.
    Coustou-Linares, V., Maddelein, M. L., Begueret, J., and Saupe, S. J. (2001) Mol. Microbiol., 42, 1325–1335.PubMedGoogle Scholar
  66. 66.
    Balguerie, A., Dos, R. S., Ritter, C., Chaignepain, S., Coulary-Salin, B., Forge, V., Bathany, K., Lascu, I., Schmitter, J. M., Riek, R., and Saupe, S. J. (2003) EMBO J., 22, 2071–2081.PubMedGoogle Scholar
  67. 67.
    Dos Reis, S., Coulary-Salin, B., Forge, V., Lascu, I., Begueret, J., and Saupe, S. J. (2002) J. Biol. Chem., 277, 5703–5706.PubMedGoogle Scholar
  68. 68.
    Maddelein, M. L., Dos Reis, S., Duvezin-Caubet, S., Coulary-Salin, B., and Saupe, S. J. (2002) Proc. Natl. Acad. Sci. USA, 99, 7402–7407.PubMedGoogle Scholar
  69. 69.
    Cox, B. (1965) Heredity, 20, 505–521.Google Scholar
  70. 70.
    Liebman, S. W., and Sherman, F. (1979) J. Bacteriol., 139, 1068–1071.PubMedGoogle Scholar
  71. 71.
    Hawthorne, D. C., and Leupold, U. (1974) Curr. Top. Microbiol. Immunol., 64, 1–47.PubMedGoogle Scholar
  72. 72.
    Inge-Vechtomov, S. G., and Adrianova, V. M. (1970) Genetika, 6, 103–115.Google Scholar
  73. 73.
    Cox, B. S., Tuite, M. F., and McLaughlin, C. S. (1988) Yeast, 4, 159–178.PubMedGoogle Scholar
  74. 74.
    Kushnirov, V. V., Ter-Avanesyan, M. D., Telckov, M. V., Surguchov, A. P., Smirnov, V. N., and Inge-Vechtomov, S. G. (1988) Gene, 66, 45–54.PubMedGoogle Scholar
  75. 75.
    Ter-Avanesyan, M. D., Dagkesamanskaya, A. R., Kushnirov, V. V., and Smirnov, V. N. (1994) Genetics, 137, 671–676.PubMedGoogle Scholar
  76. 76.
    DePace, A. H., Santoso, A., Hillner, P., and Weissman, J. S. (1998) Cell, 93, 1241–1252.PubMedGoogle Scholar
  77. 77.
    Doel, S. M., McCready, S. J., Nierras, C. R., and Cox, B. S. (1994) Genetics, 137, 659–670.PubMedGoogle Scholar
  78. 78.
    Cosson, B., Couturier, A., Chabelskaya, S., Kiktev, D., Inge-Vechtomov, S., Philippe, M., and Zhouravleva, G. (2002) Mol. Cell Biol., 22, 3301–3315.PubMedGoogle Scholar
  79. 79.
    Hoshino, S., Hosoda, N., Araki, Y., Kobayashi, T., Uchida, N., Funakoshi, Y., and Katada, T. (1999) Biochemistry (Moscow), 64, 1367–1372.Google Scholar
  80. 80.
    Hosoda, N., Kobayashi, T., Uchida, N., Funakoshi, Y., Kikuchi, Y., Hoshino, S., and Katada, T. (2003) J. Biol. Chem., 278, 38287–38291.PubMedGoogle Scholar
  81. 81.
    Bradley, M. E., and Liebman, S. W. (2004) Mol. Microbiol., 51, 1649–1659.PubMedGoogle Scholar
  82. 82.
    Liu, J. J., Sondheimer, N., and Lindquist, S. L. (2002) Proc. Natl. Acad. Sci. USA, 99, 16446–16453.PubMedGoogle Scholar
  83. 83.
    Stansfield, I., Jones, K. M., Kushnirov, V. V., Dagkesamanskaya, A. R., Poznyakovski, A. I., Paushkin, S. V., Nierras, C. R., Cox, B. S., Ter-Avanesyan, M. D., and Tuite, M. F. (1995) EMBO J., 14, 4365–4373.PubMedGoogle Scholar
  84. 84.
    Zhouravleva, G., Frolova, L., Le, G. X., Le Guellec, R., Inge-Vechtomov, S., Kisselev, L., and Philippe, M. (1995) EMBO J., 14, 4065–4072.PubMedGoogle Scholar
  85. 85.
    Ter-Avanesyan, M. D., Kushnirov, V. V., Dagkesamanskaya, A. R., Didichenko, S. A., Chernoff, Y. O., Inge-Vechtomov, S. G., and Smirnov, V. N. (1993) Mol. Microbiol., 7, 683–692.PubMedGoogle Scholar
  86. 86.
    Frolova, L., Le, G. X., Rasmussen, H. H., Cheperegin, S., Drugeon, G., Kress, M., Arman, I., Haenni, A. L., Celis, J. E., Philippe, M., et al. (1994) Nature, 372, 701–703.PubMedGoogle Scholar
  87. 87.
    Bailleul, P. A., Newnam, G. P., Steenbergen, J. N., and Chernoff, Y. O. (1999) Genetics, 153, 81–94.PubMedGoogle Scholar
  88. 88.
    Valouev, I. A., Kushnirov, V. V., and Ter-Avanesyan, M. D. (2002) Cell Motil. Cytoskeleton, 52, 161–173.PubMedGoogle Scholar
  89. 89.
    Lund, P. M., and Cox, B. S. (1981) Genet. Res., 37, 173–182.PubMedGoogle Scholar
  90. 90.
    Chernoff, Y. O., Derkach, I. L., and Inge-Vechtomov, S. G. (1993) Curr. Genet., 24, 268–270.PubMedGoogle Scholar
  91. 91.
    Derkatch, I. L., Chernoff, Y. O., Kushnirov, V. V., Inge-Vechtomov, S. G., and Liebman, S. W. (1996) Genetics, 144, 1375–1386.PubMedGoogle Scholar
  92. 92.
    Kochneva-Pervukhova, N. V., Poznyakovski, A. I., Smirnov, V. N., and Ter-Avanesyan, M. D. (1998) Curr. Genet., 34, 146–151.PubMedGoogle Scholar
  93. 93.
    Patino, M. M., Liu, J. J., Glover, J. R., and Lindquist, S. (1996) Science, 273, 622–626.PubMedGoogle Scholar
  94. 94.
    Paushkin, S. V., Kushnirov, V. V., Smirnov, V. N., and Ter-Avanesyan, M. D. (1996) EMBO J., 15, 3127–3134.PubMedGoogle Scholar
  95. 95.
    Glover, J. R., Kowal, A. S., Schirmer, E. C., Patino, M. M., Liu, J. J., and Lindquist, S. (1997) Cell, 89, 811–819.PubMedGoogle Scholar
  96. 96.
    King, C. Y., Tittmann, P., Gross, H., Gebert, R., Aebi, M., and Wuthrich, K. (1997) Proc. Natl. Acad. Sci. USA, 94, 6618–6622.PubMedGoogle Scholar
  97. 97.
    Sipe, J. D., and Cohen, A. S. (2000) J. Struct. Biol., 130, 88–98.PubMedGoogle Scholar
  98. 98.
    Nelson, R., Sawaya, M. R., Balbirnie, M., Madsen, A. O., Riekel, C., Grothe, R., and Eisenberg, D. (2005) Nature, 435, 773–778.PubMedGoogle Scholar
  99. 99.
    Paushkin, S. V., Kushnirov, V. V., Smirnov, V. N., and Ter-Avanesyan, M. D. (1997) Science, 277, 381–383.PubMedGoogle Scholar
  100. 100.
    King, C. Y., and Diaz-Avalos, R. (2004) Nature, 428, 319–323.PubMedGoogle Scholar
  101. 101.
    Tanaka, M., Chien, P., Naber, N., Cooke, R., and Weissman, J. S. (2004) Nature, 428, 323–328.PubMedGoogle Scholar
  102. 102.
    Derkatch, I. L., Bradley, M. E., Masse, S. V., Zadorsky, S. P., Polozkov, G. V., Inge-Vechtomov, S. G., and Liebman, S. W. (2000) EMBO J., 19, 1942–1952.PubMedGoogle Scholar
  103. 103.
    Sondheimer, N., and Lindquist, S. (2000) Mol. Cell, 5, 163–172.PubMedGoogle Scholar
  104. 104.
    Bradley, M. E., Edskes, H. K., Hong, J. Y., Wickner, R. B., and Liebman, S. W. (2002) Proc. Natl. Acad. Sci. USA, 99, 16392–16399.PubMedGoogle Scholar
  105. 105.
    Derkatch, I. L., Uptain, S. M., Outeiro, T. F., Krishnan, R., Lindquist, S. L., and Liebman, S. W. (2004) Proc. Natl. Acad. Sci. USA, 101, 12934–12939.PubMedGoogle Scholar
  106. 106.
    Salnikova, A. B., Kryndushkin, D. S., Smirnov, V. N., Kushnirov, V. V., and Ter-Avanesyan, M. D. (2005) J. Biol. Chem., 280, 8808–8812.PubMedGoogle Scholar
  107. 107.
    Chernoff, Y. O., Lindquist, S. L., Ono, B., Inge-Vechtomov, S. G., and Liebman, S. W. (1995) Science, 268, 880–884.PubMedGoogle Scholar
  108. 108.
    Parsell, D. A., Kowal, A. S., Singer, M. A., and Lindquist, S. (1994) Nature, 372, 475–478.PubMedGoogle Scholar
  109. 109.
    Glover, J. R., and Lindquist, S. (1998) Cell, 94, 73–82.PubMedGoogle Scholar
  110. 110.
    Lindquist, S. (1997) Cell, 89, 495–498.PubMedGoogle Scholar
  111. 111.
    Shorter, J., and Lindquist, S. (2004) Science, 304, 1793–1797.PubMedGoogle Scholar
  112. 112.
    Kushnirov, V. V., and Ter-Avanesyan, M. D. (1998) Cell, 94, 13–16.PubMedGoogle Scholar
  113. 113.
    Eaglestone, S. S., Ruddock, L. W., Cox, B. S., and Tuite, M. F. (2000) Proc. Natl. Acad. Sci. USA, 97, 240–244.PubMedGoogle Scholar
  114. 114.
    Cox, B., Ness, F., and Tuite, M. (2003) Genetics, 165, 23–33.PubMedGoogle Scholar
  115. 115.
    Ness, F., Ferreira, P., Cox, B. S., and Tuite, M. F. (2002) Mol. Cell Biol., 22, 5593–5605.PubMedGoogle Scholar
  116. 116.
    Ferreira, P. C., Ness, F., Edwards, S. R., Cox, B. S., and Tuite, M. F. (2001) Mol. Microbiol., 40, 1357–1369.PubMedGoogle Scholar
  117. 117.
    Jung, G., Jones, G., and Masison, D. C. (2002) Proc. Natl. Acad. Sci. USA, 99, 9936–9941.PubMedGoogle Scholar
  118. 118.
    Grimminger, V., Richter, K., Imhof, A., Buchner, J., and Walter, S. (2004) J. Biol. Chem., 279, 7378–7383.PubMedGoogle Scholar
  119. 119.
    Wegrzyn, R. D., Bapat, K., Newnam, G. P., Zink, A. D., and Chernoff, Y. O. (2001) Mol. Cell Biol., 21, 4656–4669.PubMedGoogle Scholar
  120. 120.
    Kushnirov, V. V., Kryndushkin, D. S., Boguta, M., Smirnov, V. N., and Ter-Avanesyan, M. D. (2000) Curr. Biol., 10, 1443–1446.PubMedGoogle Scholar
  121. 121.
    Kryndushkin, D. S., Alexandrov, I. M., Ter-Avanesyan, M. D., and Kushnirov, V. V. (2003) J. Biol. Chem., 278, 49636–49643.PubMedGoogle Scholar
  122. 122.
    Becker, J., Walter, W., Yan, W., and Craig, E. A. (1996) Mol. Cell Biol., 16, 4378–4386.PubMedGoogle Scholar
  123. 123.
    Oka, M., Nakai, M., Endo, T., Lim, C. R., Kimata, Y., and Kohno, K. (1998) J. Biol. Chem., 273, 29727–29737.PubMedGoogle Scholar
  124. 124.
    Cyr, D. M., Lu, X., and Douglas, M. G. (1992) J. Biol. Chem., 267, 20927–20931.PubMedGoogle Scholar
  125. 125.
    Newnam, G. P., Wegrzyn, R. D., Lindquist, S. L., and Chernoff, Y. O. (1999) Mol. Cell Biol., 19, 1325–1333.PubMedGoogle Scholar
  126. 126.
    Allen, K. D., Wegrzyn, R. D., Chernova, T. A., Muller, S., Newnam, G. P., Winslett, P. A., Wittich, K. B., Wilkinson, K. D., and Chernoff, Y. O. (2005) Genetics, 169, 1227–1242.PubMedGoogle Scholar
  127. 127.
    Chernoff, Y. O., Newnam, G. P., Kumar, J., Allen, K., and Zink, A. D. (1999) Mol. Cell Biol., 19, 8103–8112.PubMedGoogle Scholar
  128. 128.
    Ohba, M. (1997) FEBS Lett., 409, 307–311.PubMedGoogle Scholar
  129. 129.
    Lopez, N., Aron, R., and Craig, E. A. (2003) Mol. Biol. Cell, 14, 1172–1181.PubMedGoogle Scholar
  130. 130.
    Sondheimer, N., Lopez, N., Craig, E. A., and Lindquist, S. (2001) EMBO J., 20, 2435–2442.PubMedGoogle Scholar
  131. 131.
    Schwimmer, C., and Masison, D. C. (2002) Mol. Cell Biol., 22, 3590–3598.PubMedGoogle Scholar
  132. 132.
    Moriyama, H., Edskes, H. K., and Wickner, R. B. (2000) Mol. Cell Biol., 20, 8916–8922.PubMedGoogle Scholar
  133. 133.
    Santoso, A., Chien, P., Osherovich, L. Z., and Weissman, J. S. (2000) Cell, 100, 277–288.PubMedGoogle Scholar
  134. 134.
    Krishnan, R., and Lindquist, S. L. (2005) Nature, 435, 765–772.PubMedGoogle Scholar
  135. 135.
    Parham, S. N., Resende, C. G., and Tuite, M. F. (2001) EMBO J., 20, 2111–2119.PubMedGoogle Scholar
  136. 136.
    Osherovich, L. Z., Cox, B. S., Tuite, M. F., and Weissman, J. S. (2004) PLoS. Biol., 2, E86.PubMedGoogle Scholar
  137. 137.
    Liu, J. J., and Lindquist, S. (1999) Nature, 400, 573–576.PubMedGoogle Scholar
  138. 138.
    Kochneva-Pervukhova, N. V., Chechenova, M. B., Valouev, I. A., Kushnirov, V. V., Smirnov, V. N., and Ter-Avanesyan, M. D. (2001) Yeast, 18, 489–497.PubMedGoogle Scholar
  139. 139.
    Uptain, S. M., Sawicki, G. J., Caughey, B., and Lindquist, S. (2001) EMBO J., 20, 6236–6245.PubMedGoogle Scholar
  140. 140.
    Zhou, P., Derkatch, I. L., Uptain, S. M., Patino, M. M., Lindquist, S., and Liebman, S. W. (1999) EMBO J., 18, 1182–1191.PubMedGoogle Scholar
  141. 141.
    DePace, A. H., and Weissman, J. S. (2002) Nat. Struct. Biol., 9, 389–396.PubMedGoogle Scholar
  142. 142.
    Selkoe, D. J. (2003) Nature, 426, 900–904.PubMedGoogle Scholar
  143. 143.
    Elghetany, M. T., and Saleem, A. (1988) Stain Technol., 63, 201–212.PubMedGoogle Scholar
  144. 144.
    Sunde, M., and Blake, C. (1997) Adv. Protein Chem., 50, 123–159.PubMedGoogle Scholar
  145. 145.
    Chiti, F., Stefani, M., Taddei, N., Ramponi, G., and Dobson, C. M. (2003) Nature, 424, 805–808.PubMedGoogle Scholar
  146. 146.
    Dobson, C. M. (1999) Trends Biochem. Sci., 24, 329–332.PubMedGoogle Scholar
  147. 147.
    Dobson, C. M. (2001) Philos. Trans. R. Soc. Lond. B Biol. Sci., 356, 133–145.PubMedGoogle Scholar
  148. 148.
    Perutz, M. F., Pope, B. J., Owen, D., Wanker, E. E., and Scherzinger, E. (2002) Proc. Natl. Acad. Sci. USA, 99, 5596–5600.PubMedGoogle Scholar
  149. 149.
    Perutz, M. F., Finch, J. T., Berriman, J., and Lesk, A. (2002) Proc. Natl. Acad. Sci. USA, 99, 5591–5595.PubMedGoogle Scholar
  150. 150.
    Kishimoto, A., Hasegawa, K., Suzuki, H., Taguchi, H., Namba, K., and Yoshida, M. (2004) Biochem. Biophys. Res. Commun., 315, 739–745.PubMedGoogle Scholar
  151. 151.
    Kajava, A. V., Baxa, U., Wickner, R. B., and Steven, A. C. (2004) Proc. Natl. Acad. Sci. USA, 101, 7885–7890.PubMedGoogle Scholar
  152. 152.
    Perutz, M. F., Johnson, T., Suzuki, M., and Finch, J. T. (1994) Proc. Natl. Acad. Sci. USA, 91, 5355–5358.PubMedGoogle Scholar
  153. 153.
    Michelitsch, M. D., and Weissman, J. S. (2000) Proc. Natl. Acad. Sci. USA, 97, 11910–11915.PubMedGoogle Scholar
  154. 154.
    Enari, M., Flechsig, E., and Weissmann, C. (2001) Proc. Natl. Acad. Sci. USA, 98, 9295–9299.PubMedGoogle Scholar
  155. 155.
    Peretz, D., Williamson, R. A., Kaneko, K., Vergara, J., Leclerc, E., Schmitt-Ulms, G., Mehlhorn, I. R., Legname, G., Wormald, M. R., Rudd, P. M., Dwek, R. A., Burton, D. R., and Prusiner, S. B. (2001) Nature, 412, 739–743.PubMedGoogle Scholar
  156. 156.
    Sigurdsson, E. M., Sy, M. S., Li, R., Scholtzova, H., Kascsak, R. J., Kascsak, R., Carp, R., Meeker, H. C., Frangione, B., and Wisniewski, T. (2003) Neurosci. Lett., 336, 185–187.PubMedGoogle Scholar
  157. 157.
    White, A. R., Enever, P., Tayebi, M., Mushens, R., Linehan, J., Brandner, S., Anstee, D., Collinge, J., and Hawke, S. (2003) Nature, 422, 80–83.PubMedGoogle Scholar
  158. 158.
    Soto, C., Kascsak, R. J., Saborio, G. P., Aucouturier, P., Wisniewski, T., Prelli, F., Kascsak, R., Mendez, E., Harris, D. A., Ironside, J., Tagliavini, F., Carp, R. I., and Frangione, B. (2000) Lancet, 355, 192–197.PubMedGoogle Scholar
  159. 159.
    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. USA, 94, 10069–10074.PubMedGoogle Scholar
  160. 160.
    Crozet, C., Lin, Y. L., Mettling, C., Mourton-Gilles, C., Corbeau, P., Lehmann, S., and Perrier, V. (2004) J. Cell Sci., 117, 5591–5597.PubMedGoogle Scholar
  161. 161.
    Ma, J., and Lindquist, S. (1999) Nat. Cell Biol., 1, 358–361.PubMedGoogle Scholar
  162. 162.
    Bach, S., Talarek, N., Andrieu, T., Vierfond, J. M., Mettey, Y., Galons, H., Dormont, D., Meijer, L., Cullin, C., and Blondel, M. (2003) Nat. Biotechnol., 21, 1075–1081.PubMedGoogle Scholar
  163. 163.
    Eaglestone, S. S., Cox, B. S., and Tuite, M. F. (1999) EMBO J., 18, 1974–1981.PubMedGoogle Scholar
  164. 164.
    Si, K., Lindquist, S., and Kandel, E. R. (2003) Cell, 115, 879–891.PubMedGoogle Scholar
  165. 165.
    Si, K., Giustetto, M., Etkin, A., Hsu, R., Janisiewicz, A. M., Miniaci, M. C., Kim, J. H., Zhu, H., and Kandel, E. R. (2003) Cell, 115, 893–904.PubMedGoogle Scholar
  166. 166.
    Theis, M., Si, K., and Kandel, E. R. (2003) Proc. Natl. Acad. Sci. USA, 100, 9602–9607.PubMedGoogle Scholar
  167. 167.
    Allen, K. D., Si, K., Theis, M., and Kandel, E. R. (2005) in Abstracts of Prion Biology (Joint Cold Spring Harbor Laboratory/Wellcome Trust Conference).Google Scholar

Copyright information

© Pleiades Publishing, Ltd. 2007

Authors and Affiliations

  1. 1.Russian Cardiology Research-Industrial CenterMoscowRussia

Personalised recommendations