A hypothesis on prion disorders: Are infectious, inherited, and sporadic causes so distinct?

Brain Research Bulletin

Volumn 69, Issue 2, 2006, Pages 95-100

  Fornai, F ^a,b   Ferrucci, M ^a   Gesi, M ^a   Di Poggio, A Bandettini ^a   Giorgi, F S ^a   Biagioni, F ^b   Paparelli, A ^a  

^a UNIVERSITY OF PISA   (Italy)

^b IRCCS NEUROMED   (Italy)

Author keywords

Conformational disorders; Proteasome; Ubiquitin; Whorls

Indexed keywords

PROTEIN C FOS;

AMBIGUUS NUCLEUS; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTERIAL PRESSURE; CARDIOVASCULAR EFFECT; CONTROLLED STUDY; HEART RATE; IMMUNOREACTIVITY; LATERAL RETICULAR NUCLEUS; MALE; MECHANORECEPTOR; NONHUMAN; ONCOGENE C FOS; PRESSOR RESPONSE; PRIORITY JOURNAL; PROTEIN EXPRESSION; RAT; REFLEX; REVIEW; RHOMBENCEPHALON; SOLITARY TRACT NUCLEUS; SPLANCHNIC NERVE; STOMACH DISTENSION; THALAMUS VENTRAL NUCLEUS; VAGOTOMY; VAGUS NERVE;

BRAIN; DISEASE TRANSMISSION; INCLUSION BODIES; MODELS, NEUROLOGICAL; PRION DISEASES; PRIONS; PROTEASOME ENDOPEPTIDASE COMPLEX; PROTEIN FOLDING; PROTEIN TRANSPORT; UBIQUITIN;

EID: 33644855295     PISSN: 03619230     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.brainresbull.2005.12.005     Document Type: Review

References (71)

1. T. Alper, D.A. Haig, and M.C. Clarke The exceptionally small size of the scrapie agent Biochem. Biophys. Res. Commun. 22 1966 278 284
2. J.E. Arnold, C. Tipler, L. Laszlo, J. Hope, M. Landon, and R.J. Mayer The abnormal isoform of the prion protein accumulates in late-endosome-like organelles in scrapie-infected mouse brain J. Pathol. 176 1995 403 411
3. J.M. Bockman, S.B. Prusiner, J. Tateishi, and D.T. Kingsbury Immunoblotting of Creutzfeldt-Jakob disease prion proteins: host species-specific epitopes Ann. Neurol. 21 1987 589 595
4. D.C. Bolton, M.P. McKinley, and S.B. Prusiner Identification of a protein that purifies with the scrapie prion Science 218 1982 1309 1311
5. S. Brandner, S. Isenmann, A. Raeber, M. Fischer, A. Sailer, Y. Kobayashi, S. Marino, C. Weissmann, and A. Aguzzi Normal host prion protein necessary for scrapie-induced neurotoxicity Nature 379 1996 339 343
6. H. Bueler, A. Aguzzi, A. Sailer, R.A. Greiner, P. Autenried, M. Aguet, and C. Weissmann Mice devoid of PrP are resistant to scrapie Cell 73 1993 1339 1347
7. R.W. Carrel, and D.A. Lomas Conformational disease Lancet 350 1997 134 138
8. B. Chesebro Prion protein and the transmissible spongiform encephalopathy diseases Neuron 24 1999 503 506
9. B. Chesebro, and B. Caughey Scrapie agent replication without the prion protein? Curr. Biol. 3 1993 696 698
10. E. Cohen, and A. Taraboulos Scrapie-like prion protein accumulates in aggresomes of cyclosporin A-treated cells EMBO J. 22 2003 404 417
11. S.J. DeArmond, M.P. McKinley, R.A. Barry, M.B. Braunfeld, J.R. McColloch, and S.B. Prusiner Identification of prion amyloid filaments in scrapie-infected brain Cell 41 1985 221 235
12. S.J. DeArmond, W.C. Mobley, D.L. DeMott, R.A. Barry, J.H. Beckstead, and S.B. Prusiner Changes in the localization of brain prion proteins during scrapie infection Neurology 37 1987 1271 1280
13. F. Edenhofer, R. Rieger, M. Famulok, W. Wendler, S. Weiss, and E.-L. Winnacker Prion protein PrPc interacts with molecular chaperones of the Hsp60 family J. Virol. 70 1996 4724 4728
14. D.C. Gajdusek, C.J. Gibbs Jr., and M. Alpers Experimental transmission of a Kuru-like syndrome to chimpanzees Nature 209 1966 794 796
15. D.C. Gajdusek, and C.J. Gibbs Jr Slow, latent and temperate virus infections of the central nervous system Res. Publ. Assoc. Res. Nerv. Ment. Dis. 44 1968 254 280
16. J. Gerstmann, E. Straussler, and I.Z. Scheinker Ueber eine eigenartige hereditaer-familiaere Erkrankung des Zentralnervensystems Z. Ges. Neurol. Psychiat. 154 1936 736 762
17. R.A. Gibbons, and G.D. Hunter Nature of the scrapie agent Nature 215 1967 1041 1043
18. C.J. Gibbs Jr, D.C. Gajdusek, D.M. Asher, M.P. Alpers, E. Beck, P.M. Daniel, and W.B. Matthews Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee Science 161 1968 388 389
19. W.J. Hadlow Scrapie and Kuru Lancet 274 1959 289 290
20. A.F. Hill, S. Joiner, J. Linehan, M. Desbruslais, P.L. Lantos, and J. Collinge Species-barrier-independent prion replication in apparently resistant species Proc. Natl. Acad. Sci. U.S.A. 97 2000 10248 10253
21. K. Hsiao, H.F. Baker, T.J. Crow, M. Poulter, F. Owen, J.D. Terwilliger, D. Westaway, J. Ott, and S.B. Prusiner Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome Nature 338 1989 342 345
22. Z. Huang, J.M. Gabriel, M.A. Baldwin, R.J. Fletterick, S.B. Prusiner, and F.E. Cohen Proposed three-dimensional structure for the cellular prion protein Proc. Natl. Acad. Sci. U.S.A. 91 1994 7139 7143
23. K. Jendroska, F.P. Heinzel, M. Torkia, L. Stowring, H.A. Kretzschmar, A. Kon, A. Stern, S.B. Prusiner, and S.J. DeArmond Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity Neurology 41 1991 1482 1490
24. T. Jin, Y. Gu, G. Zanusso, M. Sy, A. Kumar, M. Cohen, P. Gambetti, and N. Singh The chaperone protein BiP binds to a mutant prion protein and mediates its degradation by the proteasome J. Biol. Chem. 275 2000 38699 38704
25. K. Kaneko, L. Zulianello, M. Scott, C.M. Cooper, A.C. Wallace, T.L. James, F.E. Cohen, and S.B. Prusiner Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation Proc. Natl. Acad. Sci. U.S.A. 94 1997 10069 10074
26. R.R. Kopito ER quality control: the cytoplasmic connection Cell 88 1997 427 430
27. C. Kurschner, and J.I. Morgan Analysis of interaction sites in homo- and heteromeric complexes containing Bcl-2 family members and the cellular prion protein Mol. Brain Res. 37 1996 249 258
28. C. Locth, B. Chesebro, R. Race, and J.M. Keith Molecular cloning and complete sequence of prion protein cDNA from mouse brain infected with the scrapie agent Proc. Natl. Acad. Sci. U.S.A. 83 1986 6372 6376
29. J. Ma, and S. Lindquist Wild-type PrP and a mutant associated with prion disease are subject to retrograde transport and proteasome degradation Proc. Natl. Acad. Sci. U.S.A. 98 2001 14955 14960
30. J. Ma, and S. Lindquist Conversion of PrP to a self-perpetuating PrP-like conformation in the cytosol Science 298 2002 1785 1788
31. J. Ma, R. Wollmann, and S. Lindquist Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol Science 298 2002 1781 1785
32. G. Mallucci, A. Dickinson, J. Linehan, P.-C. Klöhn, S. Brandner, and J. Collinge Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis Science 302 2003 871 874
33. L. Manuelidis, S. Valley, and E.E. Manuelidis Specific proteins associated with Creutzfeldt-Jakob disease and scrapie share antigenic and carbohydrate determinants Proc. Natl. Acad. Sci. U.S.A. 82 1985 4263 4267
34. C.L. Masters, D.C. Gajdusek, and C.J. Gibbs Jr. The familial occurrence of Creutzfeldt-Jakob disease and Alzheimer's disease Brain 104 1981 559 588
35. F. Meggendorfer, and Z. Gesamte Klinische und genealogische Beobachtungen bei einem Fall von spastischer Pseudosklerose Jakobs Neurol. Psychiatr. 128 1930 337 341
36. R.K. Meyer, M.P. McKinley, K.A. Bowman, M.B. Braunfeld, R.A. Barry, and S.B. Prusiner Separation and properties of cellular and scrapie prion proteins Proc. Natl. Acad. Sci. U.S.A. 83 1986 2310 2314
37. M.M. Mouradian Recent advances in the genetics and pathogenesis of Parkinson disease Neurology 58 2002 179 185
38. N. Naslavsky, R. Stein, A. Yanai, G. Friedlander, and A. Taraboulos Characterization of detergent-insoluble complexes containing the cellular prion protein and its scrapie isoform J. Biol. Chem. 272 1997 6324 6331
39. B. Oesch, D. Westaway, M. Walchli, M.P. McKinley, S.B.H. Kent, R. Aebersold, R.A. Barry, P. Tempst, D.B. Teplow, and L.E. Hood A cellular gene encodes scrapie PrP 27-30 protein Cell 40 1985 735 746
40. E. Paitel, A.C. Da Costa, D. Vilette, J. Grassi, and F. Checler Overexpression of PrPc triggers caspase 3 activation: potentiation by proteasome inhibitors and blockade by anti-PrP antibodies J. Neurochem. 83 2002 1208 1214
41. E. Paitel, R. Fahraeus, and F. Checler Cellular prion protein sensitizes neurons to apoptotic stimuli through Mdm2-regulated and p53-dependent caspase 3-like activation J. Biol. Chem. 278 2003 10061 10066
42. K.M. Pan, M. Baldwin, J. Nguyen, M. Gasset, A. Serban, D. Groth, I. Mehlhorn, Z. Huang, R.J. Fletterick, F.E. Cohen, and S.B. Prusiner Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins Proc. Natl. Acad. Sci. U.S.A. 90 1993 10962 10966
43. H.B. Parry Scrapie: a transmissible and hereditary disease of sheep Heredity 17 1962 75 105
44. I.H. Pattison D.C. Gajdusek C.J. Gibbs Jr M.P. Alpers Slow, Latent and Temperate Virus Infections 1965 U.S. Gov. Printing Office Washington 249 257
45. J.P. Peters, A. Mirinov Jr., D. Peretz, E. van Donselaar, E. Leclerc, S. Erpel, S.J. DeArmond, D.R. Burton, R.A. Williamson, M. Vey, and S.B. Prusiner Trafficking of prion proteins through a caveolae-mediated endosomal pathway J. Cell Biol. 162 2003 703 717
46. S.B. Prusiner Novel proteinaceous infectious particles cause scrapie Science 216 1982 136 144
47. S.B. Prusiner Prions Sci. Am. 251 1984 50 59
48. S.B. Prusiner Prions and neurodegenerative diseases N. Engl. J. Med. 317 1987 1571 1581
49. S.B. Prusiner Scrapie prions Annu. Rev. Microbiol. 43 1989 345 374
50. S.B. Prusiner Molecular biology of prion diseases Science 252 1991 1515 1522
51. S.B. Prusiner Prions Proc. Natl. Acad. Sci. U.S.A. 95 1998 13363 13383
52. S.B. Prusiner, D.E. Garfin, J.R. Baringer, S.P. Cochran, W.J. Hadlow, R.E. Race, and C.M. Eklund J. Stevens J. Todaro C.F. Fox Persistent Viruses 1978 Academic Press New York 591 613
53. S.B. Prusiner, D.F. Groth, D.C. Bolton, S.B. Kent, and L.E. Hood Purification and structural studies of a major scrapie prion protein Cell 38 1984 127 134
54. S.B. Prusiner, D. Groth, A. Serban, R. Koehler, D. Foster, M. Torchia, D. Burton, S.L. Yang, and S.J. DeArmond Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies Proc. Natl. Acad. Sci. U.S.A. 90 1993 10608 10612
55. R. Riek, S. Hornemann, G. Wider, M. Billeter, R. Glockshuber, and K. Wuthrich NMR structure of the mouse prion protein domain PrP(121-321) Nature 382 1996 180 182
56. A. Sailer, H. Beueler, M. Fisher, A. Aguzzi, and C. Weissmann No propagation of prions in mice devoid of PrP Cell 77 1994 967 968
57. B. Sigurdsson A chronic encephalitis of sheep: with general remarks on infections which develop slowly and some of their special characteristics Br. Vet. J. 110 1954 341 354
58. N. Singh, G. Zanusso, S.G. Chen, H. Fujioka, S. Richardson, P. Gambetti, and R.B. Petersen Prion protein aggregation reverted by low temperature in transfected cells carrying a prion protein gene mutation J. Biol. Chem. 272 1997 28461 28470
59. R.S. Sparkes, M. Simon, V.H. Cohn, R.E.K. Fournier, J. Lem, I. Klisak, C. Heinzmann, C. Blatt, M. Lucero, and T. Mohandas Assignment of the human and mouse prion protein genes to homologous chromosomes Proc. Natl. Acad. Sci. U.S.A. 83 1986 7358 7362
60. N. Stahl, D.R. Borchelt, K. Hsiao, and S.B. Prusiner Scrapie prion protein contains a phosphatidylinositol glycolipid Cell 51 1987 229 240
61. A. Taraboulos, K. Jendroska, D. Serban, S.L. Yang, S.J. DeArmond, and S.B. Prusiner Regional mapping of prion proteins in brain Proc. Natl. Acad. Sci. U.S.A. 89 1992 7620 7624
62. A. Taraboulos, A.J. Raeber, D.R. Borchelt, D. Serban, and S.B. Prusiner Synthesis and trafficking of prion proteins in cultured cells Mol. Biol. Cell 3 1992 851 863
63. S. Tenzer, L. Stoltze, B. Schonfish, J. Dengjel, M. Muller, S. Stevanovìc, H.-G. Rammensee, and H. Schild Quantitative analysis of Prion-Protein degradation by constitutive and immuno-20S proteasome indicates differences correlated with disease susceptibility J. Immunol. 172 2004 1083 1091
64. M. Watarai, S. Kim, J. Erdenebaatar, S. Makino, M. Horiuchi, T. Shirahata, S. Sakaguchi, and S. Katamine Cellular prion protein promotes Brucella infection into macrophages J. Exp. Med. 198 2003 5 17
65. M. Watarai, S. Makino, Y. Fujii, K. Okamoto, and T. Shirahata Modulation of Bruce lla-induced macropinocytosis by lipid rafts mediates intracellular replication Cell. Microbiol. 4 2002 341 355
66. M. Watarai, S. Makino, M. Michikawa, K. Yanagisawa, S. Murakami, and T. Shirahata Macrophage plasma membrane cholesterol contributes to Brucella abortus infection of mice Infect. Immun. 70 2002 4818 4825
67. W.J. Welch, and P. Gambetti Chaperoning brain diseases Nature 392 1998 23 24
68. G.A.H. Wells, A.C. Scott, C.T. Johnson, R.F. Gunning, R.D. Hancock, M. Jeffrey, M. Dawson, and R. Bradley A novel progressive spongiform encephalopathy in cattle Vet. Rec. 121 1987 419 420
69. D. Westaway, S.J. DeArmond, J. Cayetano-Canlas, D. Groth, D. Foster, S.L. Yang, M. Torchia, G.A. Carlson, and S.B. Prusiner Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins Cell 76 1994 117 129
70. Y. Yedidia, L. Horonchik, S. Tzaban, A. Yanai, and A. Taraboulos Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein EMBO J. 20 2001 5383 5391
71. G. Zanusso, R.B. Petersen, T. Jin, Y. Jing, R. Kanhoush, S. Ferrari, P. Gambetti, and N. Singh Proteasomal degradation and N-terminal protease resistance of the codon 145 mutant prion protein J. Biol. Chem. 274 1999 23396 23404