1932

Abstract

While many aspects of prion disease biology are unorthodox, perhaps the most fundamental paradox is posed by the coexistence of inherited, sporadic, and infectious forms of these diseases. Sensible molecular mechanisms for prion propagation must explain all three forms of prion diseases in a manner that is compatible with the formidable array of experimental data derived from histopathological, biochemical, biophysical, human genetic, and transgenetic studies. In this review, we explore prion disease pathogenesis initially from the perspective of an autosomal dominant inherited disease. Subsequently, we examine how an intrinsically inherited disease could present in sporadic and infectious forms. Finally, we explore the phenomenologic constraints on models of prion replication with a specific emphasis on biophysical studies of prion protein structures.

Loading

Article metrics loading...

/content/journals/10.1146/annurev.biochem.67.1.793
1998-07-01
2025-02-09
Loading full text...

Full text loading...

/deliver/fulltext/biochem/67/1/annurev.biochem.67.1.793.html?itemId=/content/journals/10.1146/annurev.biochem.67.1.793&mimeType=html&fmt=ahah

Literature Cited

  1. Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F. et al. 1989. Nature 338:342–45 [Google Scholar]
  2. Dlouhy SR, Hsiao K, Farlow MR, Foroud T, Conneally PM. et al. 1992. Nat. Genet. 1:64–67 [Google Scholar]
  3. Petersen RB, Tabaton M, Berg L, Schrank B, Torack RM. et al. 1992. Neurology 42:1859–63 [Google Scholar]
  4. Poulter M, Baker HF, Frith CD, Leach M, Lofthouse R. et al. 1992. Brain 115:675–85 [Google Scholar]
  5. Gabizon R, Rosenmann H, Meiner Z, Kahana I, Kahana E. et al. 1993. Am. J. Hum. Genet. 33:828–35 [Google Scholar]
  6. Kelly JW. 1997. Structure 5:595–600 [Google Scholar]
  7. Lee JP, Stimson ER, Ghilardi JR, Mantyh PW, Lu YA. et al. 1995. Biochemistry 34:5191–200 [Google Scholar]
  8. Colon W, Lai Z, McCutchen SL, Miroy GJ, Strang C, Kelly JW. 1996. Ciba Found. Symp. 199:228–38 [Google Scholar]
  9. Matthews BW. 1996. FASEB J. 10:35–41 [Google Scholar]
  10. Bellinger-Kawahara CG, Kempner E, Groth DF, Gabizon R, Prusiner SB. 1988. Virology 164:537–41 [Google Scholar]
  11. Huang Z, Gabriel J-M, Baldwin MA, Fletterick RJ, Prusiner SB, Cohen FE. 1994. Proc. Natl. Acad. Sci. USA 91:7139–43 [Google Scholar]
  12. Riek R, Hornemann S, Wider G, Billeter M, Glockshuber R, Wüthrich K. 1996. Nature 382:180–82 [Google Scholar]
  13. James TL, Liu H, Ulyanov NB, Farr-Jones S, Zhang H. et al. 1997. Proc. Natl. Acad. Sci. USA 94:10086–91 [Google Scholar]
  14. Mehlhorn I, Groth D, Stöckel J, Moffat B, Reilly D. et al. 1996. Biochemistry 35:5528–37 [Google Scholar]
  15. Kaneko K, Wille H, Mehlhorn I, Zhang H, Ball H. et al. 1997. J. Mol. Biol. 270:574–86 [Google Scholar]
  16. Prusiner SB. 1989. Annu. Rev. Microbiol. 43:345–74 [Google Scholar]
  17. Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P. et al. 1996. Science 274:2079–82 [Google Scholar]
  18. Hsiao KK, Groth D, Scott M, Yang S-L, Serban H. et al. 1994. Proc. Natl. Acad. Sci. USA 91:9126–30 [Google Scholar]
  19. Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M. et al. 1995. Cell 83:79–90 [Google Scholar]
  20. Telling GC, Haga T, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB. 1996. Genes Dev. 10:1736–50 [Google Scholar]
  21. Goudsmit J, Morrow CH, Asher DM, Yanagihara RT, Masters CL. et al. 1980. Neurology 30:945–50 [Google Scholar]
  22. Godec MS, Asher DM, Kozachuk WE, Masters CL, Rubi JU. et al. 1994. Neurology 44:1111–15 [Google Scholar]
  23. Prusiner SB, McKinley MP, Groth DF, Bowman KA, Mock Nl. et al. 1981. Proc. Natl. Acad. Sci. USA 78:6675–79 [Google Scholar]
  24. Prusiner SB, Groth DF, McKinley MP, Cochran SP, Bowman KA, Kasper KC. 1981. Proc. Natl. Acad. Sci. USA 78:4606–10 [Google Scholar]
  25. Prusiner SB, Groth D, Serban A, Stahl N, Gabizon R. 1993. Proc. Natl. Acad. Sci. USA 90:2793–97 [Google Scholar]
  26. Wilesmith JW, Ryan JBM, Atkinson MJ. 1991. Vet. Rec. 128:199–203 [Google Scholar]
  27. Gajdusek DC. 1977. Science 197:943–60 [Google Scholar]
  28. Prusiner SB, Cochran SP, Alpers MP. 1985. J. Infect. Dis. 152:971–78 [Google Scholar]
  29. Prusiner SB, Scott M, Foster D, Pan K-M, Groth D. et al. 1990. Cell 63:673–86 [Google Scholar]
  30. Scott M, Groth D, Foster D, Torchia M, Yang S-L. et al. 1993. Cell 73:979–88 [Google Scholar]
  31. Kocisko DA, Priola SA, Raymond GJ, Chesebro B, Lansbury PT Jr, Caughey B. 1995. Proc. Natl. Acad. Sci. USA 92:3923–27 [Google Scholar]
  32. Prusiner SB. 1997. Science 278:245–51 [Google Scholar]
  33. Kaneko K, Zulianello L, Scott M, Cooper CM, Wallace AC. et al. 1997. Proc. Natl. Acad. Sci. USA 94:10069–74 [Google Scholar]
  34. Büeler H, Aguzzi A, Sailer A, Greiner R-A, Autenried P. et al. 1993. Cell 73:1339–47 [Google Scholar]
  35. Prusiner SB, Groth D, Serban A, Koehler R, Foster D. et al. 1993. Proc. Natl. Acad. Sci. USA 90:10608–12 [Google Scholar]
  36. Manson JC, Clarke AR, McBride PA, McConnell I, Hope J. 1994. Neurodegeneration 3:331–40 [Google Scholar]
  37. Sakaguchi S, Katamine S, Shigematsu K, Nakatani A, Moriuchi R. et al. 1995. J. Virol. 69:7586–92 [Google Scholar]
  38. Oesch B, Westaway D, Wälchli M, McKinley MP, Kent SBH. et al. 1985. Cell 40:735–46 [Google Scholar]
  39. Prusiner SB, Bolton DC, Groth DF, Bowman KA, Cochran SP, McKinley MP. 1982. Biochemistry 21:6942–50 [Google Scholar]
  40. McKinley MP, Bolton DC, Prusiner SB. 1983. Cell 35:57–62 [Google Scholar]
  41. Nguyen J, Baldwin MA, Cohen FE, Prusiner SB. 1995. Biochemistry 34:4186–92 [Google Scholar]
  42. Zhang H, Kaneko K, Nguyen JT, Livshits TL, Baldwin MA. et al. 1995. J. Mol. Biol. 250:514–26 [Google Scholar]
  43. Zhang H, Stöckel J, Mehlhorn I, Groth D, Baldwin MA. et al. 1997. Biochemistry 36:3543–53 [Google Scholar]
  44. Chapman J, Ben-Israel J, Goldhammer Y, Korczyn AD. 1994. Neurology 44:1683–86 [Google Scholar]
  45. Spudich S, Mastrianni JA, Wrensch M, Gabizon R, Meiner Z. et al. 1995. Mol. Med. 1:607–13 [Google Scholar]
  46. Prusiner SB, Scott MR. 1997. Annu. Rev. Genet. 31:139–75 [Google Scholar]
  47. Büeler H, Raeber A, Sailer A, Fischer M, Aguzzi A, Weissmann C. 1994. Mol. Med. 1:19–30 [Google Scholar]
  48. Carlson GA, Ebeling C, Yang S-L, Telling G, Torchia M. et al. 1994. Proc. Natl. Acad. Sci. USA 91:5690–94 [Google Scholar]
  49. Cohen FE, Pan K-M, Huang Z, Baldwin M, Fletterick RJ, Prusiner SB. 1994. Science 264:530–31 [Google Scholar]
  50. Prusiner SB. 1982. Science 216:136–44 [Google Scholar]
  51. Prusiner SB. 1984. Sci. Am. 251:50–59 [Google Scholar]
  52. Prusiner SB. 1991. Science 252:1515–22 [Google Scholar]
  53. Prusiner SB, McKinley MP, Bowman KA, Bolton DC, Bendheim PE. et al. 1983. Cell 35:349–58 [Google Scholar]
  54. Prusiner SB, Groth DF, Bolton DC, Kent SB, Hood LE. 1984. Cell 38:127–34 [Google Scholar]
  55. Chesebro B, Race R, Wehrly K, Nishio J, Bloom M. et al. 1985. Nature 315:331–33 [Google Scholar]
  56. Sparkes RS, Simon M, Cohn VH, Fournier REK, Lem J. et al. 1986. Proc. Natl. Acad. Sci. USA 83:7358–62 [Google Scholar]
  57. Basler K, Oesch B, Scott M, Westaway D, Wälchli M. et al. 1986. Cell 46:417–28 [Google Scholar]
  58. Bamborough P, Wille H, Telling GC, Yehiely F, Prusiner SB, Cohen FE. 1996. Cold Spring Harbor Symp. Quant. Biol. 61:495–509 [Google Scholar]
  59. Alper T, Cramp WA, Haig DA, Clarke MC. 1967. Nature 214:764–66 [Google Scholar]
  60. Latarjet R, Muel B, Haig DA, Clarke MC, Alper T. 1970. Nature 227:1341–43 [Google Scholar]
  61. Diener TO, McKinley MP, Prusiner SB. 1982. Proc. Natl. Acad. Sci. USA 79:5220–24 [Google Scholar]
  62. Bellinger-Kawahara C, Cleaver JE, Diener TO, Prusiner SB. 1987. J. Virol. 61:159–66 [Google Scholar]
  63. Bellinger-Kawahara C, Diener TO, McKinley MP, Groth DF, Smith DR, Prusiner SB. 1987. Virology 160:271–74 [Google Scholar]
  64. Locht C, Chesebro B, Race R, Keith JM. 1986. Proc. Natl. Acad. Sci. USA 83:6372–76 [Google Scholar]
  65. Endo T, Groth D, Prusiner SB, Kobata A. 1989. Biochemistry 28:8380–88 [Google Scholar]
  66. Stahl N, Baldwin MA, Burlingame AL, Prusiner SB. 1990. Biochemistry 29:8879–84 [Google Scholar]
  67. Yost CS, Lopez CD, Prusiner SB, Myers RM, Lingappa VR. 1990. Nature 343:669–72 [Google Scholar]
  68. De Fea KA, Nakahara DH, Calayag MC, Yost CS, Mirels LF. et al. 1994. J. Biol. Chem. 269:16810–20 [Google Scholar]
  69. Hegde RS, Mastrianni JA, Scott MR, DeFea KA, Tremblay P. et al. 1998. Science 279:827–34 [Google Scholar]
  70. Turk E, Teplow DB, Hood LE, Prusiner SB. 1988. Eur. J. Biochem. 176:21–30 [Google Scholar]
  71. Pan K-M, Stahl N, Prusiner SB. 1992. Protein Sci. 1:1343–52 [Google Scholar]
  72. Pan K-M, Baldwin M, Nguyen J, Gasset M, Serban A. et al. 1993. Proc. Natl. Acad. Sci. USA 90:10962–66 [Google Scholar]
  73. Riek R, Hornemann S, Wider G, Glockshuber R, Wüthrich K. 1997. FEBS Lett. 413:282–88 [Google Scholar]
  74. Donne DG, Viles JH, Groth D, Mehlhorn I, James TL. et al. 1997. Proc. Natl. Acad. Sci. USA 94:13452–57 [Google Scholar]
  75. McKinley MP, Meyer R, Kenaga L, Rahbar F, Cotter R. et al. 1991. J. Virol. 65:1440–49 [Google Scholar]
  76. Stahl N, Baldwin MA, Teplow DB, Hood L, Gibson BW. et al. 1993. Biochemistry 32:1991–2002 [Google Scholar]
  77. Muramoto T, Scott M, Cohen F, Prusiner SB. 1996. Proc. Natl. Acad. Sci. USA 93:15457–62 [Google Scholar]
  78. Glenner GG, Eanes ED, Page DL. 1972. J. Histochem. Cytochem. 20:821–26 [Google Scholar]
  79. Glenner GG. 1980. N. Engl. J. Med. 302:1283–92 [Google Scholar]
  80. Bendheim PE, Barry RA, DeArmond SJ, Stites DP, Prusiner SB. 1984. Nature 310:418–21 [Google Scholar]
  81. DeArmond SJ, McKinley MP, Barry RA, Braunfeld MB, McColloch JR, Prusiner SB. 1985. Cell 41:221–35 [Google Scholar]
  82. Kitamoto T, Tateishi J, Tashima I, Takeshita I, Barry RA. et al. 1986. Ann. Neurol. 20:204–8 [Google Scholar]
  83. Roberts GW, Lofthouse R, Allsop D, Landon M, Kidd M. et al. 1988. Neurology 38:1534–40 [Google Scholar]
  84. Caughey BW, Dong A, Bhat KS, Ernst D, Hayes SF, Caughey WS. 1991. Biochemistry 30:7672–80 [Google Scholar]
  85. Safar J, Roller PP, Gajdusek DC, Gibbs CJ Jr. 1993. J. Biol. Chem. 268:20276–84 [Google Scholar]
  86. Pergami P, Jaffe H, Safar J. 1996. Anal. Biochem. 236:63–73 [Google Scholar]
  87. Huang Z, Prusiner SB, Cohen FE. 1996. Folding Des. 1:13–19 [Google Scholar]
  88. Williamson RA, Peretz D, Smorodinsky N, Bastidas R, Serban H. et al. 1996. Proc. Natl. Acad. Sci. USA 93:7279–82 [Google Scholar]
  89. Barry RA, Prusiner SB. 1986. J. Infect. Dis. 154:518–21 [Google Scholar]
  90. Kascsak RJ, Rubenstein R, Merz PA, Tonna-DeMasi M, Fersko R. et al. 1987. J. Virol. 61:3688–93 [Google Scholar]
  91. Peretz D, Williamson RA, Matsunaga Y, Serban H, Pinilla C. et al. 1997. J. Mol. Biol. 273:614–22 [Google Scholar]
  92. Korth C, Stierli B, Streit P, Moser M, Schaller O. et al. 1997. Nature 389:74–77 [Google Scholar]
  93. Pattison IH. 1965. In Slow, Latent and Temperate Virus Infections, NINDB Monogr. 2, ed. DC Gajdusek, CJ Gibbs Jr, MP Alpers 249–57 Washington, DC: US Gov. Print. Off
  94. Scott M, Foster D, Mirenda C, Serban D, Coufal F. et al. 1989. Cell 59:847–57 [Google Scholar]
  95. Scott MR, Köhler R, Foster D, Prusiner SB. 1992. Protein Sci. 1:986–97 [Google Scholar]
  96. Kocisko DA, Come JH, Priola SA, Chesebro B, Raymond GJ. et al. 1994. Nature 370:471–74 [Google Scholar]
  97. Kaneko K, Peretz D, Pan K-M, Blochberger T, Wille H. et al. 1995. Proc. Natl. Acad. Sci. USA 32:11160–64 [Google Scholar]
  98. Rogers M, Yehiely F, Scott M, Prusiner SB. 1993. Proc. Natl. Acad. Sci. USA 90:3182–86 [Google Scholar]
  99. Fischer M, Rülicke T, Raeber A, Sailer A, Moser M. et al. 1996. EMBO J. 15:1255–64 [Google Scholar]
  100. Kitamoto T, Iizuka R, Tateishi J. 1993. Biochem. Biophys. Res. Commun. 192:525–31 [Google Scholar]
  101. Hornemann S, Glockshuber R. 1996. J. Mol. Biol. 262:614–19 [Google Scholar]
  102. Haraguchi T, Fisher S, Olofsson S, Endo T, Groth D. et al. 1989. Arch. Biochem. Biophys. 274:1–13 [Google Scholar]
  103. Chen SG, Teplow DB, Parchi P, Teller JK, Gambetti P, Autilio-Gambetti L. 1995. J. Biol. Chem. 270:19173–80 [Google Scholar]
  104. Taraboulos A, Scott M, Semenov A, Avrahami D, Laszlo L, Prusiner SB. 1995. J. Cell Biol. 129:121–32 [Google Scholar]
  105. Gasset M, Baldwin MA, Lloyd D, Gabriel J-M, Holtzman DM. et al. 1992. Proc. Natl. Acad. Sci. USA 89:10940–44 [Google Scholar]
  106. Heller J, Kolbert AC, Larsen R, Ernst M, Bekker T. et al. 1996. Protein Sci. 5:1655–61 [Google Scholar]
  107. Forloni G, Angeretti N, Chiesa R, Monzani E, Salmona M. et al. 1993. Nature 362:543–46 [Google Scholar]
  108. De Gioia L, Selvaggini C, Ghibaudi E, Diomede L, Bugiani O. et al. 1994. J. Biol. Chem. 269:7859–62 [Google Scholar]
  109. Brown DR, Schmidt B, Kretzschmar HA. 1996. Nature 380:345–47 [Google Scholar]
  110. Güntert P, Braun W, Wüthrich K. 1991. J. Mol. Biol. 217:517–30 [Google Scholar]
  111. Schätzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB. 1995. J. Mol. Biol. 245:362–74 [Google Scholar]
  112. Doh-ura K, Tateishi J, Sasaki H, Kitamoto T, Sakaki Y. 1989. Biochem. Biophys. Res. Commun. 163:974–79 [Google Scholar]
  113. Hsiao KK, Cass C, Schellenberg GD, Bird T, Devine-Gage E. et al. 1991. Neurology 41:681–84 [Google Scholar]
  114. Mastrianni JA, Curtis MT, Oberholtzer JC, Da Costa MM, DeArmond S. et al. 1995. Neurology 45:2042–50 [Google Scholar]
  115. Scott MR, Nguyen O, Stöckel J, Tatzelt J, DeArmond SJ. et al. 1997. Protein Sci. 6 (Suppl. 1):84 [Google Scholar]
  116. Medori R, Tritschler H-J, LeBlanc A, Villare F, Manetto V. et al. 1992. N. Engl. J. Med. 326:444–49 [Google Scholar]
  117. Goldfarb LG, Petersen RB, Tabaton M, Brown P, LeBlanc AC. et al. 1992. Science 258:806–8 [Google Scholar]
  118. Westaway D, Zuliani V, Cooper CM, Da Costa M, Neuman S. et al. 1994. Genes Dev. 8:959–69 [Google Scholar]
  119. Hunter N, Goldmann W, Benson G, Foster JD, Hope J. 1993. J. Gen. Virol. 74:1025–31 [Google Scholar]
  120. Goldmann W, Hunter N, Smith G, Foster J, Hope J. 1994. J. Gen. Virol. 75:989–95 [Google Scholar]
  121. Belt PBGM, Muileman IH, Schreuder BEC, Ruijter JB, Gielkens ALJ, Smits MA. 1995. J. Gen. Virol. 76:509–17 [Google Scholar]
  122. Clousard C, Beaudry P, Elsen JM, Milan D, Dussaucy M. et al. 1995. J. Gen. Virol. 76:2097–101 [Google Scholar]
  123. Ikeda T, Horiuchi M, Ishiguro N, Muramatsu Y, Kai-Uwe GD, Shinagawa M. 1995. J. Gen. Virol. 76:2577–81 [Google Scholar]
  124. Hunter N, Moore L, Hosie BD, Dingwall WS, Greig A. 1997. Vet. Rec. 140:59–63 [Google Scholar]
  125. Hunter N, Cairns D, Foster JD, Smith G, Goldmann W, Donnelly K. 1997. Nature 386:137 [Google Scholar]
  126. O'Rourke KI, Holyoak GR, Clark WW, Mickelson JR, Wang S. et al. 1997. J. Gen. Virol. 78:975–78 [Google Scholar]
  127. Kitamoto T, Tateishi J. 1994. Philos. Trans. R. Soc. London Ser. B 343:391–98 [Google Scholar]
  128. Shibuya S, Higuchi J, Shin R-W, Tateishi J, Kitamoto T. 1998. Lancet 351:419 [Google Scholar]
  129. Riek R, Hornemann S, Wider G, Glockshuber R, Wüthrich K. 1997. FEBS Lett. 413:282–88 [Google Scholar]
  130. Donne DG, Viles JH, Groth D, Mehlhorn I, James TL. et al. 1997. Proc. Natl. Acad. Sci. USA 94:13452–57 [Google Scholar]
  131. Stöckel J, Safar J, Wallace AC, Cohen FE, Prusiner SB. 1998. Biochemistry. In press [Google Scholar]
  132. Hornshaw MP, McDermott JR, Candy JM, Lakey JH. 1995. Biochem. Biophys. Res. Commun. 214:993–99 [Google Scholar]
  133. Hornshaw MP, McDermott JR, Candy JM. 1995. Biochem. Biophys. Res. Commun. 207:621–29 [Google Scholar]
  134. Miura T, Hori-i A, Takeuchi H. 1996. FEBS Lett. 396:248–52 [Google Scholar]
  135. Brown DR, Qin K, Herms JW, Madlung A, Manson J. et al. 1997. Nature 390:684–87 [Google Scholar]
  136. Bruce ME, Dickinson AG. 1987. J. Gen. Virol. 68:79–89 [Google Scholar]
  137. Dickinson AG, Outram GW. 1988. In Novel Infectious Agents and the Central Nervous System. Ciba Found. Symp. 135, ed. G Bock, J Marsh 63–83 Chichester, UK: Wiley
  138. Ridley RM, Baker HF. 1996. Neurodegeneration 5:219–31 [Google Scholar]
  139. Pattison IH, Millson GC. 1961. J. Comp. Pathol. 71:101–8 [Google Scholar]
  140. Fraser H, Dickinson AG. 1973. J. Comp. Pathol. 83:29–40 [Google Scholar]
  141. Dickinson AG, Fraser HG. 1977. In Slow Virus Infections of the Central Nervous System, ed. V ter Meulen, M Katz 3–14 New York: Springer-Verlag
  142. Kimberlin RH, Cole S, Walker CA. 1987. J. Gen. Virol. 68:1875–81 [Google Scholar]
  143. Scott MR, Groth D, Tatzelt J, Torchia M, Tremblay P. et al. 1997. J. Virol. 7:9032–44 [Google Scholar]
  144. Bessen RA, Marsh RF. 1994. J. Virol. 68:7859–68 [Google Scholar]
  145. Monari L, Chen SG, Brown P, Parchi P, Petersen RB. et al. 1994. Proc. Natl. Acad. Sci. USA 91:2839–42 [Google Scholar]
  146. Bennett MJ, Schlunegger MP, Eisenberg D. 1995. Protein Sci. 4:2455–68 [Google Scholar]
  147. Anfinsen CB. 1973. Science 181:223–30 [Google Scholar]
  148. Chan HS, Dill KA. 1996. Proteins 24:335–44 [Google Scholar]
  149. Pearlman DA, Case DA, Caldwell J, Ross WS, Cheatham TE III. et al. 1995. AMBER 4. 1. San Francisco: Univ. Calif
/content/journals/10.1146/annurev.biochem.67.1.793
Loading
/content/journals/10.1146/annurev.biochem.67.1.793
Loading

Data & Media loading...

  • Article Type: Introduction
This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error