The relationship between the 20S proteasomes and prion-mediated neurodegenerations: Potential therapeutic opportunities

Apoptosis

Volumn 15, Issue 11, 2010, Pages 1322-1335

  Cecarini, Valentina ^a   Bonfili, Laura ^a   Cuccioloni, Massimiliano ^a   Mozzicafreddo, Matteo ^a   Angeletti, Mauro ^a   Eleuteri, Anna Maria ^a  

^a UNIVERSITY OF CAMERINO   (Italy)

Author keywords

20S proteasomes; Apoptosis; Prion diseases; Therapeutic strategies

Indexed keywords

AMPHOTERICIN B; ANTIMONIOTUNGSTATE; CARRAGEENAN; CONGO RED; CURCUMIN; DEXTRAN SULFATE; OLIGONUCLEOTIDE PHOSPHOROTHIOATE; PENTOSAN POLYSULFATE; PHTHALOCYANINE DERIVATIVE; PORPHYRIN DERIVATIVE; PRION PROTEIN; PROTEASOME; SURAMIN;

ALPHA HELIX; ARTICLE; ASTROCYTOSIS; BOVINE SPONGIFORM ENCEPHALOPATHY; CELL CYCLE PROGRESSION; CLINICAL FEATURE; COMPETITIVE INHIBITION; CREUTZFELDT JAKOB DISEASE; DISEASE ASSOCIATION; ENDOPLASMIC RETICULUM; FATAL FAMILIAL INSOMNIA; GENETIC POLYMORPHISM; GENETIC SUSCEPTIBILITY; GERSTMANN STRAUSSLER SCHEINKER SYNDROME; HOMOZYGOSITY; HUMAN; NERVE DEGENERATION; NEUROPROTECTION; NEUROTOXICITY; NEUROTRANSMISSION; NONHUMAN; PATHOGENESIS; POINT MUTATION; PRION DISEASE; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN DEGRADATION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN METABOLISM; PROTEIN STRUCTURE; PROTEIN TARGETING; SYNAPTIC TRANSMISSION; UBIQUITINATION;

ANIMALS; HUMANS; NEURODEGENERATIVE DISEASES; PRION DISEASES; PRIONS; PROTEASOME ENDOPEPTIDASE COMPLEX;

MAMMALIA;

EID: 78650023648     PISSN: 13608185     EISSN: 1573675X     Source Type: Journal    
DOI: 10.1007/s10495-010-0480-1     Document Type: Article

References (159)

1. AL Goldberg 2003 Protein degradation and protection against misfolded or damaged proteins Nature 426 895 899 1:CAS:528:DC%2BD3sXpvVGmtbc%3D 14685250
2. DC Rubinsztein 2006 The roles of intracellular protein-degradation pathways in neurodegeneration Nature 443 780 786 1:CAS:528:DC%2BD28XhtVyktbbN 17051204
3. L Bedford D Hay A Devoy, et al. 2008 Depletion of 26S proteasomes in mouse brain neurons causes neurodegeneration and Lewy-like inclusions resembling human pale bodies J Neurosci 28 8189 8198 1:CAS:528:DC%2BD1cXhtVaktLjN 18701681
4. M Kristiansen P Deriziotis DE Dimcheff, et al. 2007 Disease-associated prion protein oligomers inhibit the 26S proteasome Mol Cell 26 175 188 1:CAS:528:DC%2BD2sXltFCqtbk%3D 17466621
5. CA Ross MA Poirier 2004 Protein aggregation and neurodegenerative disease Nat Med 10 Suppl S10 S17 15272267
6. SB Prusiner 1982 Novel proteinaceous infectious particles cause scrapie Science 216 136 144 1:CAS:528:DyaL38XhsFyqtro%3D 6801762
7. SB Prusiner 1998 Prions Proc Natl Acad Sci USA 95 13363 13383 1:CAS:528:DyaK1cXnsVGhsbY%3D 9811807
8. A Aguzzi M Polymenidou 2004 Mammalian prion biology: one century of evolving concepts Cell 116 313 327 1:CAS:528:DC%2BD2cXhtVals7g%3D 14744440
9. J Collinge 2001 Prion diseases of humans and animals: their causes and molecular basis Annu Rev Neurosci 24 519 550 1:CAS:528:DC%2BD3MXls1Shs7s%3D 11283320
10. B Oesch D Westaway M Walchli, et al. 1985 A cellular gene encodes scrapie PrP 27-30 protein Cell 40 735 746 1:CAS:528:DyaL2MXitFKhsLw%3D 2859120
11. P Barbanti G Fabbrini M Salvatore, et al. 1996 Polymorphism at codon 129 or codon 219 of PRNP and clinical heterogeneity in a previously unreported family with Gerstmann-Straussler-Scheinker disease (PrP-P102L mutation) Neurology 47 734 741 1:CAS:528:DyaK28XmtFWisbg%3D 8797472
12. P Brown LG Goldfarb WR McCombie, et al. 1992 Atypical Creutzfeldt-Jakob disease in an American family with an insert mutation in the PRNP amyloid precursor gene Neurology 42 422 427 1:CAS:528:DyaK38Xitleisbg%3D 1736177
13. LG Goldfarb P Brown E Mitrova, et al. 1991 Creutzfeldt-Jacob disease associated with the PRNP codon 200Lys mutation: an analysis of 45 families Eur J Epidemiol 7 477 486 1:STN:280:DyaK387gsVaqtg%3D%3D 1684755
14. HS Lee N Sambuughin L Cervenakova, et al. 1999 Ancestral origins and worldwide distribution of the PRNP 200K mutation causing familial Creutzfeldt-Jakob disease Am J Hum Genet 64 1063 1070 1:STN:280: DyaK1M7pt1emsA%3D%3D 10090891
15. K Young CK Jones P Piccardo, et al. 1995 Gerstmann-Straussler-Scheinker disease with mutation at codon 102 and methionine at codon 129 of PRNP in previously unreported patients Neurology 45 1127 1134 1:STN:280: DyaK2Mzgtlaktg%3D%3D 7783876
16. S Mead 2006 Prion disease genetics Eur J Hum Genet 14 273 281 1:CAS:528:DC%2BD28Xhs1eqsbc%3D 16391566
17. M Bruce A Chree I McConnell J Foster G Pearson H Fraser 1994 Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier Philos Trans R Soc Lond B 343 405 411 1:STN:280: DyaK2czhs1Oisw%3D%3D
18. PT Lansbury HA Lashuel 2006 A century-old debate on protein aggregation and neurodegeneration enters the clinic Nature 443 774 779 1:CAS:528: DC%2BD28XhtVyktbbM 17051203
19. JB Martin 1999 Molecular basis of the neurodegenerative disorders N Engl J Med 340 1970 1980 1:CAS:528:DyaK1MXkt1yitrs%3D 10379022
20. FE Cohen KM Pan Z Huang M Baldwin RJ Fletterick SB Prusiner 1994 Structural clues to prion replication Science 264 530 531 1:CAS:528: DyaK2cXkslOrsrg%3D 7909169
21. C Weissmann AJ Raeber F Montrasio, et al. 2001 Prions and the lymphoreticular system Philos Trans R Soc Lond B 356 177 184 1:CAS:528:DC%2BD3MXjt1Wjtro%3D
22. HB Parry 1960 Scrapie: a transmissible hereditary disease of sheep Nature 185 441 443 1:STN:280:DC%2BD1cjis1CksA%3D%3D 18990804
23. A Fediaevsky E Morignat C Ducrot D Calavas 2009 A case-control study on the origin of atypical scrapie in sheep, France Emerg Infect Dis 15 710 718 19402956
24. S Gubbins KM McIntyre 2009 Prevalence of sheep infected with classical scrapie in Great Britain, 1993-2007 Epidemiol Infect 137 787 791 1:STN:280:DC%2BD1M3psValsg%3D%3D 19351431
25. KB Stevens VJ Del Rio Vilas J Guitian 2009 Classical sheep scrapie in Great Britain: spatial analysis and identification of environmental and farm-related risk factors BMC Vet Res 5 33 19737376
26. G Vaccari G Scavia M Sala, et al. 2009 Protective effect of the AT137RQ and ARQK176 PrP allele against classical scrapie in Sarda breed sheep Vet Res 40 19 19171116
27. K Tsunoda T Namikawa K Sato, et al. 2009 Prion protein polymorphisms and estimation of risk of scrapie in East Asian sheep Biochem Genet 48 13 25
28. W Goldmann 2008 PrP genetics in ruminant transmissible spongiform encephalopathies Vet Res 39 30 18284908
29. PJ Bosque 2002 Bovine spongiform encephalopathy, chronic wasting disease, scrapie, and the threat to humans from prion disease epizootics Curr Neurol Neurosci Rep 2 488 495 12359101
30. GP Concepcion MP David EA Padlan 2005 Why don't humans get scrapie from eating sheep? A possible explanation based on secondary structure predictions Med Hypotheses 64 919 924 1:CAS:528:DC%2BD2MXisVClsrs%3D 15780485
31. GA Wells AC Scott CT Johnson, et al. 1987 A novel progressive spongiform encephalopathy in cattle Vet Rec 121 419 420 1:STN:280:DyaL1c7gtFahug%3D%3D 3424605
32. JW Wilesmith JB Ryan MJ Atkinson 1991 Bovine spongiform encephalopathy: epidemiological studies on the origin Vet Rec 128 199 203 1:STN:280: DyaK3M3htleqsQ%3D%3D 1823120
33. J Collinge M Rossor 1996 A new variant of prion disease Lancet 347 916 917 1:STN:280:DyaK287nvVKrtg%3D%3D 8598749
34. RA Bessen RF Marsh 1992 Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent J Virol 66 2096 2101 1:CAS:528:DyaK38Xitlaqu7Y%3D 1347795
35. RF Marsh WJ Hadlow 1992 Transmissible mink encephalopathy Rev Sci Tech 11 539 550 1:STN:280:DyaK38zhsVCgtA%3D%3D 1535524
36. GR Pearson TJ Gruffydd-Jones JM Wyatt, et al. 1991 Feline spongiform encephalopathy Vet Rec 128 532 1:STN:280:DyaK3MzislGrsw%3D%3D 1866888
37. ES Williams S Young 1980 Chronic wasting disease of captive mule deer: a spongiform encephalopathy J Wildl Dis 16 89 98 1:STN:280:DyaL3c7osFWqtg%3D%3D 7373730
38. RC Angers SR Browning TS Seward, et al. 2006 Prions in skeletal muscles of deer with chronic wasting disease Science 311 1117 1:CAS:528: DC%2BD28XhvFSjtLw%3D 16439622
39. P Parchi A Giese S Capellari, et al. 1999 Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects Ann Neurol 46 224 233 1:STN:280:DyaK1MzntFCrug%3D%3D 10443888
40. A Dagvadorj RB Petersen HS Lee, et al. 2002 Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy Ann Neurol 52 355 359 1:CAS:528:DC%2BD38Xns1amtro%3D 12205650
41. MD Spencer RS Knight RG Will 2002 First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological features BMJ 324 1479 1482 12077031
42. JC Watts A Balachandran D Westaway 2006 The expanding universe of prion diseases PLoS Pathog 2 e26 16609731
43. RA Shakir 1998 New variant Creutzfeldt-Jakob disease J Neurol Sci 155 138 140 1:STN:280:DyaK1c3itVOhtA%3D%3D 9562257
44. RG Will JW Ironside M Zeidler, et al. 1996 A new variant of Creutzfeldt-Jakob disease in the UK Lancet 347 921 925 1:STN:280: DyaK287nvVKruw%3D%3D 8598754
45. F Gammie 1994 Weak evidence for human link to BSE infected meat Nature 367 402 1:STN:280:DyaK2c7ltVKjtw%3D%3D 8107788
46. HM Schatzl F Wopfner S Gilch A von Brunn G Jager 1997 Is codon 129 of prion protein polymorphic in human beings but not in animals? Lancet 349 1603 1604 1:CAS:528:DyaK2sXjvV2lsb0%3D 9174569
47. DC Gajdusek V Zigas 1957 Degenerative disease of the central nervous system in New Guinea; the endemic occurrence of kuru in the native population N Engl J Med 257 974 978 1:STN:280:DyaG1c%2Fis1GltA%3D%3D 13483871
48. DC Gajdusek V Zigas 1959 Kuru; clinical, pathological and epidemiological study of an acute progressive degenerative disease of the central nervous system among natives of the Eastern Highlands of New Guinea Am J Med 26 442 469 1:STN:280:DyaG1M%2Fls1Gnug%3D%3D 13626997
49. A Nieto LG Goldfarb P Brown, et al. 1991 Codon 178 mutation in ethnically diverse Creutzfeldt-Jakob disease families Lancet 337 622 623 1:STN:280:DyaK3M7lsFKisg%3D%3D 1671983
50. CL Masters DC Gajdusek CJ Gibbs Jr 1981 Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Straussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies Brain 104 559 588 1:STN:280:DyaL38%2FgtFeruw%3D%3D 6791762
51. LG Goldfarb P Brown A Vrbovska, et al. 1992 An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann-Straussler-Scheinker family J Neurol Sci 111 189 194 1:CAS:528:DyaK3sXpvFGkuw%3D%3D 1431985
52. J Collinge MS Palmer 1992 Prion diseases Curr Opin Genet Dev 2 448 454 1:CAS:528:DyaK3sXitVSisg%3D%3D 1504620
53. SB Prusiner 1991 Molecular biology of prion diseases Science 252 1515 1522 1:CAS:528:DyaK3MXksVKqsbo%3D 1675487
54. JS Griffith 1967 Self-replication and scrapie Nature 215 1043 1044 1:STN:280:DyaF1c%2Fht1amtw%3D%3D 4964084
55. D Westaway SB Prusiner 1986 Conservation of the cellular gene encoding the scrapie prion protein Nucleic Acids Res 14 2035 2044 1:CAS:528: DyaL28XhvVSrtrg%3D 2870469
56. J Manson JD West V Thomson P McBride MH Kaufman J Hope 1992 The prion protein gene: a role in mouse embryogenesis? Development 115 117 122 1:CAS:528:DyaK38Xlt1Gku78%3D 1353438
57. R Riek S Hornemann G Wider M Billeter R Glockshuber K Wuthrich 1996 NMR structure of the mouse prion protein domain PrP(121-321) Nature 382 180 182 1:CAS:528:DyaK28XktlSkurk%3D 8700211
58. L Calzolai DA Lysek DR Perez P Guntert K Wuthrich 2005 Prion protein NMR structures of chickens, turtles, and frogs Proc Natl Acad Sci USA 102 651 655 1:CAS:528:DC%2BD2MXis1Cqsrk%3D 15647366 (Pubitemid 40282720)
59. DA Lysek C Schorn LG Nivon, et al. 2005 Prion protein NMR structures of cats, dogs, pigs, and sheep Proc Natl Acad Sci USA 102 640 645 1:CAS:528:DC%2BD2MXis1Cqsrs%3D 15647367
60. R Linden VR Martins MA Prado M Cammarota I Izquierdo RR Brentani 2008 Physiology of the prion protein Physiol Rev 88 673 728 1:CAS:528: DC%2BD1cXls1KhsLo%3D 18391177
61. NR Cashman R Loertscher J Nalbantoglu, et al. 1990 Cellular isoform of the scrapie agent protein participates in lymphocyte activation Cell 61 185 192 1:CAS:528:DyaK3cXitFCrsLY%3D 1969332
62. T Kitamoto T Muramoto S Mohri K Doh-Ura J Tateishi 1991 Abnormal isoform of prion protein accumulates in follicular dendritic cells in mice with Creutzfeldt-Jakob disease J Virol 65 6292 6295 1:CAS:528:DyaK3MXmsFeisLY%3D 1681118
63. M Horiuchi N Yamazaki T Ikeda N Ishiguro M Shinagawa 1995 A cellular form of prion protein (PrPC) exists in many non-neuronal tissues of sheep J Gen Virol 76 Pt 10 2583 2587 1:CAS:528:DyaK2MXosVGltLY%3D 7595362
64. J Herms T Tings S Gall, et al. 1999 Evidence of presynaptic location and function of the prion protein J Neurosci 19 8866 8875 1:CAS:528: DyaK1MXms1ahtbw%3D 10516306
65. J Collinge MA Whittington KC Sidle, et al. 1994 Prion protein is necessary for normal synaptic function Nature 370 295 297 1:CAS:528: DyaK2cXltVGqt74%3D 8035877
66. X Roucou AC LeBlanc 2005 Cellular prion protein neuroprotective function: implications in prion diseases J Mol Med 83 3 11 1:CAS:528: DC%2BD2MXisFeqsA%3D%3D 15645198
67. DT Lin J Jodoin M Baril CG Goodyer AC Leblanc 2008 Cytosolic prion protein is the predominant anti-Bax prion protein form: exclusion of transmembrane and secreted prion protein forms in the anti-Bax function Biochim Biophys Acta 1783 2001 2012 1:CAS:528:DC%2BD1cXhtV2isL3J 18590778
68. X Roucou PN Giannopoulos Y Zhang J Jodoin CG Goodyer A LeBlanc 2005 Cellular prion protein inhibits proapoptotic Bax conformational change in human neurons and in breast carcinoma MCF-7 cells Cell Death Differ 12 783 795 1:CAS:528:DC%2BD2MXltFKjsbw%3D 15846375
69. M Turu M Slevin P Ethirajan, et al. 2008 The normal cellular prion protein and its possible role in angiogenesis Front Biosci 13 6491 6500 1:CAS:528:DC%2BD1cXnsVOit7Y%3D 18508675
70. N Vassallo J Herms 2003 Cellular prion protein function in copper homeostasis and redox signalling at the synapse J Neurochem 86 538 544 1:CAS:528:DC%2BD3sXlvF2ntb4%3D 12859667
71. M Hodak R Chisnell W Lu J Bernholc 2009 Functional implications of multistage copper binding to the prion protein Proc Natl Acad Sci USA 106 11576 11581 1:CAS:528:DC%2BD1MXptVKjsbY%3D 19561303
72. H Bueler M Fischer Y Lang, et al. 1992 Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein Nature 356 577 582 1:CAS:528:DyaK38XisFehsLk%3D 1373228
73. JC Manson AR Clarke ML Hooper L Aitchison I McConnell J Hope 1994 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal Mol Neurobiol 8 121 127 1:CAS:528:DyaK2cXmt1Olu74%3D 7999308
74. GR Mallucci S Ratte EA Asante, et al. 2002 Post-natal knockout of prion protein alters hippocampal CA1 properties, but does not result in neurodegeneration EMBO J 21 202 210 1:CAS:528:DC%2BD38XhsVSgu7g%3D 11823413
75. DA Harris 2003 Trafficking, turnover and membrane topology of PrP Br Med Bull 66 71 85 1:CAS:528:DC%2BD3sXnvVeqsbg%3D 14522850
76. T Haraguchi S Fisher S Olofsson, et al. 1989 Asparagine-linked glycosylation of the scrapie and cellular prion proteins Arch Biochem Biophys 274 1 13 1:CAS:528:DyaL1MXlvV2kurc%3D 2505674
77. N Stahl DR Borchelt K Hsiao SB Prusiner 1987 Scrapie prion protein contains a phosphatidylinositol glycolipid Cell 51 229 240 1:CAS:528: DyaL1cXovF2nsw%3D%3D 2444340
78. K Abid C Soto 2006 The intriguing prion disorders Cell Mol Life Sci 63 2342 2351 1:CAS:528:DC%2BD28XhtFyrtrvO 16927029
79. D Sarnataro A Caputo P Casanova, et al. 2009 Lipid rafts and clathrin cooperate in the internalization of PrP in epithelial FRT cells PLoS One 4 e5829 19503793
80. M Vey S Pilkuhn H Wille, et al. 1996 Subcellular colocalization of the cellular and scrapie prion proteins in caveolae-like membranous domains Proc Natl Acad Sci USA 93 14945 14949 1:CAS:528:DyaK28XnsVOrsrc%3D 8962161
81. SL Shyng KL Moulder A Lesko DA Harris 1995 The N-terminal domain of a glycolipid-anchored prion protein is essential for its endocytosis via clathrin-coated pits J Biol Chem 270 14793 14800 1:CAS:528:DyaK2MXmsVShsr4%3D 7782345
82. RE Griffiths KJ Heesom DJ Anstee 2007 Normal prion protein trafficking in cultured human erythroblasts Blood 110 4518 4525 1:CAS:528:DC%2BD1cXot1ag 17827389
83. B Vincent E Paitel P Saftig, et al. 2001 The disintegrins ADAM10 and TACE contribute to the constitutive and phorbol ester-regulated normal cleavage of the cellular prion protein J Biol Chem 276 37743 37746 1:CAS:528: DC%2BD3MXotFOrsbk%3D 11477090
84. DR Taylor NM Hooper 2006 The prion protein and lipid rafts Mol Membr Biol 23 89 99 1:CAS:528:DC%2BD28Xis1Citb0%3D 16611584
85. TJ Pinheiro 2006 The role of rafts in the fibrillization and aggregation of prions Chem Phys Lipids 141 66 71 1:CAS:528:DC%2BD28XltVKnsbc%3D 16647049
86. B Caughey GJ Raymond 1991 The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive J Biol Chem 266 18217 18223 1:CAS:528:DyaK3MXltlejtr4%3D 1680859
87. J Tatzelt KF Winklhofer 2004 Folding and misfolding of the prion protein in the secretory pathway Amyloid 11 162 172 1:CAS:528:DC%2BD2cXnslSnt7s%3D 15523918
88. DR Borchelt A Taraboulos SB Prusiner 1992 Evidence for synthesis of scrapie prion proteins in the endocytic pathway J Biol Chem 267 16188 16199 1:CAS:528:DyaK38XlsVygs7o%3D 1353761
89. Z Marijanovic A Caputo V Campana C Zurzolo 2009 Identification of an intracellular site of prion conversion PLoS Pathog 5 e1000426 19424437
90. H Bueler A Aguzzi A Sailer, et al. 1993 Mice devoid of PrP are resistant to scrapie Cell 73 1339 1347 1:CAS:528:DyaK3sXkvFaltbk%3D 8100741
91. K Basler B Oesch M Scott, et al. 1986 Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene Cell 46 417 428 1:CAS:528: DyaL28XlsVOgt70%3D 2873895
92. FE Cohen SB Prusiner 1998 Pathologic conformations of prion proteins Annu Rev Biochem 67 793 819 1:CAS:528:DyaK1cXlsFOmsbc%3D 9759504
93. AL Horwich JS Weissman 1997 Deadly conformations-protein misfolding in prion disease Cell 89 499 510 1:CAS:528:DyaK2sXjsVShsLY%3D 9160742
94. JD Harper PT Lansbury Jr 1997 Models of amyloid seeding in Alzheimer's disease and scrapie: mechanistic truths and physiological consequences of the time-dependent solubility of amyloid proteins Annu Rev Biochem 66 385 407 1:CAS:528:DyaK2sXktFOhsrc%3D 9242912
95. M Stefani CM Dobson 2003 Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution J Mol Med 81 678 699 1:CAS:528:DC%2BD3sXovVOgsb8%3D 12942175
96. R Kayed E Head JL Thompson, et al. 2003 Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis Science 300 486 489 1:CAS:528:DC%2BD3sXivFyms7k%3D 12702875
97. B Caughey PT Lansbury 2003 Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders Annu Rev Neurosci 26 267 298 1:CAS:528:DC%2BD3sXntFSisL0%3D 12704221
98. V Novitskaya OV Bocharova I Bronstein IV Baskakov 2006 Amyloid fibrils of mammalian prion protein are highly toxic to cultured cells and primary neurons J Biol Chem 281 13828 13836 1:CAS:528:DC%2BD28Xkt1ymurY%3D 16554307
99. Y Bounhar Y Zhang CG Goodyer A LeBlanc 2001 Prion protein protects human neurons against Bax-mediated apoptosis J Biol Chem 276 39145 39149 1:CAS:528:DC%2BD3MXnvVems7g%3D 11522774
100. X Roucou Q Guo Y Zhang CG Goodyer AC LeBlanc 2003 Cytosolic prion protein is not toxic and protects against Bax-mediated cell death in human primary neurons J Biol Chem 278 40877 40881 1:CAS:528:DC%2BD3sXotFWgsbk%3D 12917444
101. BH Kim HG Lee JK Choi, et al. 2004 The cellular prion protein (PrPC) prevents apoptotic neuronal cell death and mitochondrial dysfunction induced by serum deprivation Brain Res Mol Brain Res 124 40 50 1:CAS:528: DC%2BD2cXjt1Cgsrk%3D 15093684
102. DA Harris HL True 2006 New insights into prion structure and toxicity Neuron 50 353 357 1:CAS:528:DC%2BD28XkvFSgsLc%3D 16675391
103. L Westergard HM Christensen DA Harris 2007 The cellular prion protein (PrP(C)): its physiological function and role in disease Biochim Biophys Acta 1772 629 644 1:CAS:528:DC%2BD2sXmtlWjurs%3D 17451912
104. P Deriziotis SJ Tabrizi 2008 Prions and the proteasome Biochim Biophys Acta 1782 713 722 1:CAS:528:DC%2BD1cXhsVCntb%2FP 18644436
105. ED Werner JL Brodsky AA McCracken 1996 Proteasome-dependent endoplasmic reticulum-associated protein degradation: an unconventional route to a familiar fate Proc Natl Acad Sci USA 93 13797 13801 1:CAS:528:DyaK28Xnt1GmsLY%3D 8943015
106. Amici M, Eleuteri AM (2007) Structure and function of the 20S proteasomes. In: Di Napoli M, Wojcik C (eds) The ubiquitin proteasome system in the central nervous system: from physiology to pathology. Nova Biomedical Books, Happauge
107. A Orsi L Fioriti R Chiesa R Sitia 2006 Conditions of endoplasmic reticulum stress favor the accumulation of cytosolic prion protein J Biol Chem 281 30431 30438 1:CAS:528:DC%2BD28XhtVCrsLzL 16908519
108. J Ma S Lindquist 2001 Wild-type PrP and a mutant associated with prion disease are subject to retrograde transport and proteasome degradation Proc Natl Acad Sci USA 98 14955 14960 1:CAS:528:DC%2BD38Xptlym 11742063
109. Y Yedidia L Horonchik S Tzaban A Yanai A Taraboulos 2001 Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein EMBO J 20 5383 5391 1:CAS:528:DC%2BD38XjtVSqtg%3D%3D 11574470
110. M Dron F Dandoy-Dron MK Farooq Salamat H Laude 2009 Proteasome inhibitors promote the sequestration of PrPSc into aggresomes within the cytosol of prion-infected CAD neuronal cells J Gen Virol 90 2050 2060 1:CAS:528: DC%2BD1MXpvVyktLc%3D 19339478
111. E Cohen A Taraboulos 2003 Scrapie-like prion protein accumulates in aggresomes of cyclosporin A-treated cells EMBO J 22 404 417 1:CAS:528: DC%2BD3sXitlKlu7g%3D 12554642
112. J Ma R Wollmann S Lindquist 2002 Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol Science 298 1781 1785 1:CAS:528: DC%2BD38XovFKms7g%3D 12386337
113. J Ma S Lindquist 2002 Conversion of PrP to a self-perpetuating PrPSc-like conformation in the cytosol Science 298 1785 1788 1:CAS:528: DC%2BD38XovFKms7Y%3D 12386336
114. SC Kang DR Brown M Whiteman, et al. 2004 Prion protein is ubiquitinated after developing protease resistance in the brains of scrapie-infected mice J Pathol 203 603 608 1:CAS:528:DC%2BD2cXkt12itLw%3D 15095484
115. M Kristiansen MJ Messenger PC Klohn, et al. 2005 Disease-related prion protein forms aggresomes in neuronal cells leading to caspase activation and apoptosis J Biol Chem 280 38851 38861 1:CAS:528:DC%2BD2MXht1SktLbM 16157591
116. M Amici V Cecarini M Cuccioloni, et al. 2010 Interplay between 20S proteasomes and prion proteins in scrapie disease J Neurosci Res 88 191 201 1:CAS:528:DC%2BD1MXhtl2qtbzI 19658198
117. O Chakrabarti RS Hegde 2009 Functional depletion of mahogunin by cytosolically exposed prion protein contributes to neurodegeneration Cell 137 1136 1147 1:CAS:528:DC%2BD1MXps1eiu7g%3D 19524515
118. BR Whatley L Li LS Chin 2008 The ubiquitin-proteasome system in spongiform degenerative disorders Biochim Biophys Acta 1782 700 712 1:CAS:528:DC%2BD1cXhsVCntb%2FO 18790052
119. V Campana D Sarnataro C Fasano P Casanova S Paladino C Zurzolo 2006 Detergent-resistant membrane domains but not the proteasome are involved in the misfolding of a PrP mutant retained in the endoplasmic reticulum J Cell Sci 119 433 442 1:CAS:528:DC%2BD28Xit1Klsbg%3D 16443748
120. B Drisaldi RS Stewart C Adles, et al. 2003 Mutant PrP is delayed in its exit from the endoplasmic reticulum, but neither wild-type nor mutant PrP undergoes retrotranslocation prior to proteasomal degradation J Biol Chem 278 21732 21743 1:CAS:528:DC%2BD3sXksVemsbo%3D 12663673
121. CP Dohm P Kermer M Bahr 2008 Aggregopathy in neurodegenerative diseases: mechanisms and therapeutic implication Neurodegener Dis 5 321 338 1:CAS:528:DC%2BD1cXhtV2jurbF 18309232
122. B Caughey RE Race 1992 Potent inhibition of scrapie-associated PrP accumulation by Congo red J Neurochem 59 768 771 1:CAS:528:DyaK38Xlt1CjtLg%3D 1352803
123. B Caughey D Ernst RE Race 1993 Congo red inhibition of scrapie agent replication J Virol 67 6270 6272 1:CAS:528:DyaK3sXms1Khsbg%3D 8103804
124. S Caspi M Halimi A Yanai SB Sasson A Taraboulos R Gabizon 1998 The anti-prion activity of Congo red. Putative mechanism J Biol Chem 273 3484 3489 1:CAS:528:DyaK1cXhtVymsrY%3D 9452472
125. R Demaimay J Harper H Gordon D Weaver B Chesebro B Caughey 1998 Structural aspects of Congo red as an inhibitor of protease-resistant prion protein formation J Neurochem 71 2534 2541 1:CAS:528:DyaK1cXns1eitbw%3D 9832153
126. O Milhavet A Mange D Casanova S Lehmann 2000 Effect of Congo red on wild-type and mutated prion proteins in cultured cells J Neurochem 74 222 230 1:CAS:528:DC%2BD3cXlt12i 10617123
127. A Mange N Nishida O Milhavet HE McMahon D Casanova S Lehmann 2000 Amphotericin B inhibits the generation of the scrapie isoform of the prion protein in infected cultures J Virol 74 3135 3140 1:CAS:528:DC%2BD3cXhvVejt7s%3D 10708429
128. S Webb T Lekishvili C Loeschner, et al. 2007 Mechanistic insights into the cure of prion disease by novel antiprion compounds J Virol 81 10729 10741 1:CAS:528:DC%2BD2sXhtV2gtbbL 17652397
129. H Rudyk S Vasiljevic RM Hennion CR Birkett J Hope IH Gilbert 2000 Screening Congo red and its analogues for their ability to prevent the formation of PrP-res in scrapie-infected cells J Gen Virol 81 1155 1164 1:CAS:528:DC%2BD3cXisVersr8%3D 10725446
130. G Poli W Ponti G Carcassola, et al. 2003 In vitro evaluation of the anti-prionic activity of newly synthesized Congo red derivatives Arzneimittelforschung 53 875 888 1:CAS:528:DC%2BD2cXntVCkug%3D%3D 14750496
131. RH Kimberlin CA Walker 1986 Suppression of scrapie infection in mice by heteropolyanion 23, dextran sulfate, and some other polyanions Antimicrob Agents Chemother 30 409 413 1:CAS:528:DyaL28Xls1CmsLc%3D 2430521
132. AS Strimpakos RA Sharma 2008 Curcumin: preventive and therapeutic properties in laboratory studies and clinical trials Antioxid Redox Signal 10 511 545 1:CAS:528:DC%2BD1cXmt1yksQ%3D%3D 18370854
133. GM Cole B Teter SA Frautschy 2007 Neuroprotective effects of curcumin Adv Exp Med Biol 595 197 212 17569212
134. B Caughey LD Raymond GJ Raymond L Maxson J Silveira GS Baron 2003 Inhibition of protease-resistant prion protein accumulation in vitro by curcumin J Virol 77 5499 5502 1:CAS:528:DC%2BD3sXjtFyrs7w%3D 12692251
135. RE Ali SI Rattan 2006 Curcumin's biphasic hormetic response on proteasome activity and heat-shock protein synthesis in human keratinocytes Ann N Y Acad Sci 1067 394 399 1:CAS:528:DC%2BD28Xms1Okt7s%3D 16804017
136. A Ciechanover P Brundin 2003 The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg Neuron 40 427 446 1:CAS:528:DC%2BD3sXosFCqs70%3D 14556719
137. DA Kocisko A Vaillant KS Lee, et al. 2006 Potent antiscrapie activities of degenerate phosphorothioate oligonucleotides Antimicrob Agents Chemother 50 1034 1044 1:CAS:528:DC%2BD28XisFamtb4%3D 16495266
138. B Caughey K Brown GJ Raymond GE Katzenstein W Thresher 1994 Binding of the protease-sensitive form of PrP (prion protein) to sulfated glycosaminoglycan and congo red [corrected] J Virol 68 2135 2141 1:CAS:528:DyaK2cXis1OisLs%3D 7511169
139. RH Kimberlin CA Walker 1979 Antiviral compound effective against experimental scrapie Lancet 2 591 592 1:STN:280:DyaE1M3ktlKhsg%3D%3D 89602
140. RH Kimberlin CA Walker 1983 The antiviral compound HPA-23 can prevent scrapie when administered at the time of infection Arch Virol 78 9 18 1:CAS:528:DyaL2cXivFentg%3D%3D 6686005
141. B Ehlers H Diringer 1984 Dextran sulphate 500 delays and prevents mouse scrapie by impairment of agent replication in spleen J Gen Virol 65 Pt 8 1325 1330 1:CAS:528:DyaL2cXltVelu7w%3D 6205119
142. K Doh-ura K Ishikawa I Murakami-Kubo, et al. 2004 Treatment of transmissible spongiform encephalopathy by intraventricular drug infusion in animal models J Virol 78 4999 5006 1:CAS:528:DC%2BD2cXjvFeqsr0%3D 15113880
143. GJ Raymond EA Olsen KS Lee, et al. 2006 Inhibition of protease-resistant prion protein formation in a transformed deer cell line infected with chronic wasting disease J Virol 80 596 604 1:CAS:528:DC%2BD28XivVOltg%3D%3D 16378962
144. R Gabizon Z Meiner M Halimi SA Ben-Sasson 1993 Heparin-like molecules bind differentially to prion-proteins and change their intracellular metabolic fate J Cell Physiol 157 319 325 1:CAS:528:DyaK2cXivVCksA%3D%3D 7901226
145. O Schonberger L Horonchik R Gabizon D Papy-Garcia D Barritault A Taraboulos 2003 Novel heparan mimetics potently inhibit the scrapie prion protein and its endocytosis Biochem Biophys Res Commun 312 473 479 1:CAS:528:DC%2BD3sXptVelurs%3D 14637161
146. WS Caughey LD Raymond M Horiuchi B Caughey 1998 Inhibition of protease-resistant prion protein formation by porphyrins and phthalocyanines Proc Natl Acad Sci USA 95 12117 12122 1:CAS:528:DyaK1cXmsl2ktb0%3D 9770449 (Pubitemid 28483728)
147. SA Priola A Raines WS Caughey 2000 Porphyrin and phthalocyanine antiscrapie compounds Science 287 1503 1506 1:CAS:528:DC%2BD3cXhsV2qu7Y%3D 10688802
148. L Soler P Caffrey HE McMahon 2008 Effects of new amphotericin analogues on the scrapie isoform of the prion protein Biochim Biophys Acta 1780 1162 1167 1:CAS:528:DC%2BD1cXhtVShsrbE 18691635
149. G Forloni S Iussich T Awan, et al. 2002 Tetracyclines affect prion infectivity Proc Natl Acad Sci USA 99 10849 10854 1:CAS:528:DC%2BD38Xmt1Chtb4%3D 12149459
150. F Tagliavini G Forloni L Colombo, et al. 2000 Tetracycline affects abnormal properties of synthetic PrP peptides and PrP(Sc) in vitro J Mol Biol 300 1309 1322 1:CAS:528:DC%2BD3cXkvFaku7w%3D 10903871
151. F Gillardon A Kloss M Berg, et al. 2007 The 20S proteasome isolated from Alzheimer's disease brain shows post-translational modifications but unchanged proteolytic activity J Neurochem 101 1483 1490 1:CAS:528:DC%2BD2sXnvVant78%3D 17286585
152. KM Sakamoto KB Kim A Kumagai F Mercurio CM Crews RJ Deshaies 2001 Protacs: chimeric molecules that target proteins to the Skp1-Cullin-F box complex for ubiquitination and degradation Proc Natl Acad Sci USA 98 8554 8559 1:CAS:528:DC%2BD3MXls1Wisbk%3D 11438690
153. KM Sakamoto 2005 Chimeric molecules to target proteins for ubiquitination and degradation Methods Enzymol 399 833 847 1:CAS:528:DC%2BD2sXlvFymtbk%3D 16338398
154. JS Schneekloth Jr FN Fonseca M Koldobskiy, et al. 2004 Chemical genetic control of protein levels: selective in vivo targeted degradation J Am Chem Soc 126 3748 3754 1:CAS:528:DC%2BD2cXhvVegur0%3D 15038727
155. Cardoso I, Merlini G, Saraiva MJ (2003) 4'-iodo-4'-deoxydoxorubicin and tetracyclines disrupt transthyretin amyloid fibrils in vitro producing noncytotoxic species: screening for TTR fibril disrupters. FASEB J 17:803-809
156. Merlini G, Ascari E, Amboldi N, et al (1995) Interaction of the anthracycline 4'-iodo-4'-deoxydoxorubicin with amyloid fibrils: inhibition of amyloidogenesis. Proc Natl Acad Sci USA 92:2959-2963
157. Tagliavini F, McArthur RA, Canciani B, et al (1997) Effectiveness of anthracycline against experimental prion disease in Syrian hamsters. Science 276:1119-1122
158. Yang F, Lim GP, Begum AN, et al (2005) Curcumin inhibits formation of amyloid beta oligomers and fibrils, binds plaques, and reduces amyloid in vivo. J Biol Chem 280:5892-5901
159. Ono K, Hasegawa K, Naiki H, Yamada M (2004) Curcumin has potent anti-amyloidogenic effects for Alzheimer's beta-amyloid fibrils in vitro. J Neurosci Res 75:742-750