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Chapter 24 : Prion Diseases
Author:
Christopher J. Silva1
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Affiliations:
1: Foodborne Contaminants Research Unit, Agricultural Research Service, Western Regional Research Center, USDA, Albany, CA 94710
Content Type: Monograph
Publication Year:
2009
Category: Clinical Microbiology
Book DOI:
10.1128/9781555815486
Chapter DOI:
10.1128/9781555815486.ch24
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Abstract:
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are unlike any other malady. Prion diseases have long, largely asymptomatic incubation periods. Although the name would suggest all TSEs are acquired, in fact most prion diseases have no apparent cause and are called sporadic. TSEs are caused by a novel contagion once referred to as a slow virus and now called a prion. Scrapie is the oldest known TSE; it was first described in Europe at least 250 years ago. The presence of amyloid in the brains of kuru victims suggested a relationship with a much broader category of diseases. The relative resistance of a prion to proteinase K (PK) digestion is strain dependent. Worldwide, familial prion diseases account for 10 to 15% of all prion diseases. Kuru was eliminated when local authorities intervened to persuade members of the Fore tribe to stop ritual cannibalism. Diagnosing a prion disease is, by necessity, a diagnosis by elimination, since they are extremely rare. TSEs comprise a set of rare fatal neurological diseases found in many mammals and include such human diseases as Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker (GSS), fatal familial insomnia (FFI), and kuru.
Citation:
Silva C.
2009.
Prion Diseases,
p 425-442.
In
Fratamico P, Smith J, Brogden K (ed),
Sequelae and Long-Term Consequences of Infectious Diseases.
ASM Press, Washington, DC.
doi: 10.1128/9781555815486.ch24
Figures
Prion Diseases
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Citation:
Silva C.
2009.
Prion Diseases,
p 425-442.
In
Fratamico P, Smith J, Brogden K (ed),
Sequelae and Long-Term Consequences of Infectious Diseases.
ASM Press, Washington, DC.
doi: 10.1128/9781555815486.ch24
/content/10.1128/9781555815486.ch24.ch24fig01
Figure 1.
Version of mouse PrPC (note that the sugar antennae are represented in their most complex form) (
145
).
10.1128/9781555815486/ch24fig1_thmb.gif
10.1128/9781555815486/ch24fig1.gif
Figure 1.
Version of mouse PrPC (note that the sugar antennae are represented in their most complex form) ( 145 ).
Citation:
Silva C.
2009.
Prion Diseases,
p 425-442.
In
Fratamico P, Smith J, Brogden K (ed),
Sequelae and Long-Term Consequences of Infectious Diseases.
ASM Press, Washington, DC.
doi: 10.1128/9781555815486.ch24
/content/10.1128/9781555815486.ch24.ch24fig02
Figure 2.
Catalog of polymorphisms found in human PrPC. Amino acid codes: A, alanine; D, aspartic acid; E, glutamic acid; F, phenylalanine; G, glycine; H, histidine; I, isoleucone), K, lysine; L, leucine; M, methionine; N, asparagine; P, proline; Q, glutamine; R, arginine; S, serine; T, threonine; V, valine; Y, tyrosine (
114
,
145
).
10.1128/9781555815486/ch24fig2_thmb.gif
10.1128/9781555815486/ch24fig2.gif
Figure 2.
Catalog of polymorphisms found in human PrPC. Amino acid codes: A, alanine; D, aspartic acid; E, glutamic acid; F, phenylalanine; G, glycine; H, histidine; I, isoleucone), K, lysine; L, leucine; M, methionine; N, asparagine; P, proline; Q, glutamine; R, arginine; S, serine; T, threonine; V, valine; Y, tyrosine ( 114 , 145 ).
Citation:
Silva C.
2009.
Prion Diseases,
p 425-442.
In
Fratamico P, Smith J, Brogden K (ed),
Sequelae and Long-Term Consequences of Infectious Diseases.
ASM Press, Washington, DC.
doi: 10.1128/9781555815486.ch24
References
/content/book/10.1128/9781555815486.ch24
1.
Aguzzi, A.,, F. Baumann, and, J. Bremer. 2008. The prion’s elusive reason for being. Annu. Rev. Neurosci. 31: 439– 477.
2.
Aguzzi, A.,, M. Heikenwalder, and, M. Polymenidou. 2007. Insights into prion strains and neurotoxicity. Nat. Rev. Mol. Cell Biol. 8: 552– 561.
3.
Alper, T.,, D. A. Haig, and, M. C. Clarke. 1966. The exceptionally small size of the scrapie agent. Biochem. Biophys. Res. Commun. 22: 278– 284.
4.
Alperovitch, A.,, I. Zerr,, M. Pocchiari,, E. Mitrova,, J. de Pedro Cuesta,, I. Hegyi,, S. Collins,, H. Kretzschmar,, C. van Duijn, and, R. G. Will. 1999. Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 353: 1673– 1674.
5.
Anderson, C. A.,, P. Bosque,, C. M. Filley,, D. B. Arciniegas,, B. K. Kleinschmidt-Demasters,, W. J. Pape, and, K. L. Tyler. 2007. Colorado surveillance program for chronic wasting disease transmission to humans: lessons from 2 highly suspicious but negative cases. Arch. Neurol. 64: 439– 441.
6.
Reference deleted.
7.
Reference deleted.
8.
Reference deleted.
9.
Anonymous. 2007. Fourth case of transfusion-associated vCJD infection in the United Kingdom. Euro. Surveill. 12: E070118.4.
10.
Reference deleted.
11.
Anonymous. 2006. Third case of vCJD reported in the United States. Euro. Surveill. 11: E061207.2.
12.
Basler, K.,, B. Oesch,, M. Scott,, D. Westaway,, M. Walchli,, D. F. Groth,, M. P. McKinley,, S. B. Prusiner, and, C. Weissmann. 1986. Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell 46: 417– 428.
13.
Bateman, D.,, D. Hilton,, S. Love,, M. Zeidler,, J. Beck, and, J. Collinge. 1995. Sporadic Creutzfeldt-Jakob disease in a 18-year-old in the UK. Lancet 346: 1155– 1156.
14.
Baxter, H. C.,, G. A. Campbell,, A. G. Whittaker,, A. C. Jones,, A. Aitken,, A. H. Simpson,, M. Casey,, L. Bountiff,, L. Gibbard, and, R. L. Baxter. 2005. Elimination of transmissible spongiform encephalopathy infectivity and decontamination of surgical instruments by using radio-frequency gas-plasma treatment. J. Gen. Virol. 86: 2393– 2399.
15.
Belay, E. D.,, P. Gambetti,, L. B. Schonberger,, P. Parchi,, D. R. Lyon,, S. Capellari,, J. H. McQuiston,, K. Bradley,, G. Dowdle,, J. M. Crutcher, and, C. R. Nichols. 2001. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch. Neurol. 58: 1673– 1678.
16.
Belay, E. D.,, J. J. Sejvar,, W. J. Shieh,, S. T. Wiersma,, W. Q. Zou,, P. Gambetti,, S. Hunter,, R. A. Maddox,, L. Crockett,, S. R. Zaki, and, L. B. Schonberger. 2005. Variant Creutzfeldt-Jakob disease death, United States. Emerg. Infect. Dis. 11: 1351– 1354.
17.
Bellinger-Kawahara, C. G.,, E. Kempner,, D. Groth,, R. Gabizon, and, S. B. Prusiner. 1988. Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da. Virology 164: 537– 541.
18.
Bellon, A.,, W. Seyfert-Brandt,, W. Lang,, H. Baron,, A. Groner, and, M. Vey. 2003. Improved conformation-dependent immunoassay: suitability for human prion detection with enhanced sensitivity. J. Gen. Virol. 84: 1921– 1925.
19.
Bendheim, P. E.,, H. R. Brown,, R. D. Rudelli,, L. J. Scala,, N. L. Goller,, G. Y. Wen,, R. J. Kascsak,, N. R. Cashman, and, D. C. Bolton. 1992. Nearly ubiquitous tissue distribution of the scrapie agent precursor protein. Neurology 42: 149– 156.
20.
Bernoulli, C.,, J. Siegfried,, G. Baumgartner,, F. Regli,, T. Rabinowicz,, D. C. Gajdusek, and, C. J. Gibbs, Jr. 1977. Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet i: 478– 479.
21.
Bessen, R. A.,, and R. F. Marsh. 1992. Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent. J. Virol. 66: 2096– 2101.
22.
Bone, I.,, L. Belton,, A. S. Walker, and, J. Darbyshire. 2008. Intraventricular pentosan polysulphate in human prion diseases: an observational study in the UK. Eur. J. Neurol. 15: 458– 464.
23.
Borg, M. 2006. Symptomatic myoclonus. Neurophysiol. Clin. 36: 309– 318.
24.
Brandel, J. P.,, M. Preece,, P. Brown,, E. Croes,, J. L. Laplanche,, Y. Agid,, R. Will, and, A. Alperovitch. 2003. Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK. Lancet 362: 128– 130.
25.
Britton, T. C.,, S. al-Sarraj,, C. Shaw,, T. Campbell, and, J. Collinge. 1995. Sporadic Creutzfeldt-Jakob disease in a 16-year-old in the UK. Lancet 346: 1155.
26.
Brown, P.,, J. P. Brandel,, M. Preece, and, T. Sato. 2006. Iatrogenic Creutzfeldt-Jakob disease: the waning of an era. Neurology 67: 389– 393.
27.
Brown, P.,, C. J. Gibbs, Jr.,, P. Rodgers-Johnson,, D. M. Asher,, M. P. Sulima,, A. Bacote,, L. G. Goldfarb, and, D. C. Gajdusek. 1994. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann. Neurol. 35: 513– 529.
28.
Brown, P.,, M. Preece,, J. P. Brandel,, T. Sato,, L. McShane,, I. Zerr,, A. Fletcher,, R. G. Will,, M. Pocchiari,, N. R. Cashman,, J. H. d’Aignaux,, L. Cervenakova,, J. Fradkin,, L. B. Schonberger, and, S. J. Collins. 2000. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 55: 1075– 1081.
29.
Brown, P.,, E. H. Rau,, P. Lemieux,, B. K. Johnson,, A. E. Bacote, and, D. C. Gajdusek. 2004. Infectivity studies of both ash and air emissions from simulated incineration of scrapie-contaminated tissues. Environ. Sci. Technol. 38: 6155– 6160.
30.
Bruce, M. E. 1993. Scrapie strain variation and mutation. Br. Med. Bull. 49: 822– 838.
31.
Bruce, M. E.,, A. Chree,, I. McConnell,, J. Foster,, G. Pearson, and, 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.
32.
Bruce, M. E.,, I. McConnell,, R. G. Will, and, J. W. Ironside. 2001. Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues. Lancet 358: 208– 209.
33.
Bruce, M. E.,, R. G. Will,, J. W. Ironside,, I. McConnell,, D. Drummond,, A. Suttie,, L. McCardle,, A. Chree,, J. Hope,, C. Birkett,, S. Cousens,, H. Fraser, and, C. J. Bostock. 1997. Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent. Nature 389: 498– 501.
34.
Bueler, H.,, A. Aguzzi,, A. Sailer,, R. A. Greiner,, P. Autenried,, M. Aguet, and, C. Weissmann. 1993. Mice devoid of PrP are resistant to scrapie. Cell 73: 1339– 1347.
35.
Bueler, H.,, M. Fischer,, Y. Lang,, H. Bluethmann,, H. P. Lipp,, S. J. DeArmond,, S. B. Prusiner,, M. Aguet, and, C. Weissmann. 1992. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 356: 577– 582.
36.
Casalone, C.,, G. Zanusso,, P. Acutis,, S. Ferrari,, L. Capucci,, F. Tagliavini,, S. Monaco, and, M. Caramelli. 2004. Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc. Natl. Acad. Sci. USA 101: 3065– 3070.
37.
Centers for Disease Control and Prevention. 2007. Section IV, Laboratory biosafety level criteria. In J. Y. Richmond and, R. W. McKinney (ed.), Biosafety in Microbiological and Biomedical Laboratories (BMBL), 5th ed. U.S. Government Printing Office, Washington, DC. http://www.cdc.gov/od/ohs/biosfty/bmbl5/bmbl5toc.htm. Accessed 1 August 2008.
38.
Centers for Disease Control and Prevention. 2007. Section VIII-H, Prion diseases. In J. Y. Richmond and, R. W. McKinney (ed.), Biosafety in Microbiological and Biomedical Laboratories (BMBL), 5th ed. U.S. Government Printing Office, Washington, DC. http://www.cdc.gov/od/ohs/biosfty/bmbl5/bmbl5toc.htm. Accessed 16 March 2009.
39.
Centers for Disease Control and Prevention. 2004. Creutzfeldt-Jakob disease not related to a common venue—New Jersey, 1995-2004. MMWR Morb. Mortal. Wkly. Rep. 53: 392– 396.
40.
Centers for Disease Control and Prevention. 1985. Fatal degenerative neurologic disease in patients who received pituitary-derived human growth hormone. MMWR Morb. Mortal. Wkly. Rep. 34: 359– 360, 365– 366.
41.
Centers for Disease Control and Prevention. 2003. Fatal degenerative neurologic illnesses in men who participated in wild game feasts—Wisconsin, 2002. MMWR Morb. Mortal. Wkly. Rep. 52: 125– 127.
42.
Centers for Disease Control and Prevention. 1987. Rapidly progressive dementia in a patient who received a cadaveric dura mater graft. MMWR Morb. Mortal. Wkly. Rep. 36: 49– 50, 55.
43.
Cervenakova, L.,, L. G. Goldfarb,, R. Garruto,, H. S. Lee,, D. C. Gajdusek, and, P. Brown. 1998. Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease. Proc. Natl. Acad. Sci. USA 95: 13239– 13241.
44.
Chatelain, J.,, F. Cathala,, P. Brown,, S. Raharison,, L. Court, and, D. C. Gajdusek. 1981. Epidemiologic comparisons between Creutzfeldt-Jakob disease and scrapie in France during the 12-year period 1968-1979. J. Neurol. Sci. 51: 329– 337.
45.
Chesebro, B.,, R. Race,, K. Wehrly,, J. Nishio,, M. Bloom,, D. Lechner,, S. Bergstrom,, K. Robbins,, L. Mayer,, J. M. Keith,, C. Garon, and, A. Haase. 1985. Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain. Nature 315: 331– 333.
46.
Chesebro, B.,, M. Trifilo,, R. Race,, K. Meade-White,, C. Teng,, R. LaCasse,, L. Raymond,, C. Favara,, G. Baron,, S. Priola,, B. Caughey,, E. Masliah, and, M. Oldstone. 2005. Anchorless prion protein results in infectious amyloid disease without clinical scrapie. Science 308: 1435– 1439.
47.
Chiti, F.,, and C. M. Dobson. 2006. Protein misfolding, functional amyloid, and human disease. Annu. Rev. Biochem. 75: 333– 366.
48.
Choi, C. J.,, A. Kanthasamy,, V. Anantharam, and, A. G. Kanthasamy. 2006. Interaction of metals with prion protein: possible role of divalent cations in the pathogenesis of prion diseases. Neurotoxicology 27: 777– 787.
49.
Clarke, P.,, R. G. Will, and, A. C. Ghani. 2007. Is there the potential for an epidemic of variant Creutzfeldt-Jakob disease via blood transfusion in the UK? J. R. Soc. Interface 4: 675– 684.
50.
Collinge, J. 2005. Molecular neurology of prion disease. J. Neurol. Neurosurg. Psychiatry 76: 906– 919.
51.
Collinge, J.,, and A. R. Clarke. 2007. A general model of prion strains and their pathogenicity. Science 318: 930– 936.
52.
Collinge, J.,, M. S. Palmer, and, A. J. Dryden. 1991. Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease. Lancet 337: 1441– 1442.
53.
Collinge, J.,, M. S. Palmer,, K. C. Sidle,, I. Gowland,, R. Medori,, J. Ironside, and, P. Lantos. 1995. Transmission of fatal familial insomnia to laboratory animals. Lancet 346: 569– 570.
54.
Collinge, J.,, K. C. Sidle,, J. Meads,, J. Ironside, and, A. F. Hill. 1996. Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature 383: 685– 690.
55.
Collins, S. J.,, P. Sanchez-Juan,, C. L. Masters,, G. M. Klug,, C. van Duijn,, A. Poleggi,, M. Pocchiari,, S. Almonti,, N. Cuadrado-Corrales,, J. de Pedro-Cuesta,, H. Budka,, E. Gelpi,, M. Glatzel,, M. Tolnay,, E. Hewer,, I. Zerr,, U. Heinemann,, H. A. Kretszchmar,, G. H. Jansen,, E. Olsen,, E. Mitrova,, A. Alperovitch,, J. P. Brandel,, J. Mackenzie,, K. Murray, and, R. G. Will. 2006. Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease. Brain 129: 2278– 2287.
56.
D’Aignaux, J. H.,, S. N. Cousens,, N. Delasnerie-Laupretre,, J. P. Brandel,, D. Salomon,, J. L. Laplanche,, J. J. Hauw, and, A. Alperovitch. 2002. Analysis of the geographical distribution of sporadic Creutzfeldt-Jakob disease in France between 1992 and 1998. Int. J. Epidemiol. 31: 490– 495.
57.
Darby, S. C.,, S. W. Kan,, R. J. Spooner,, P. L. Giangrande,, F. G. Hill,, C. R. Hay,, C. A. Lee,, C. A. Ludlam, and, M. Williams. 2007. Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood 110: 815– 825.
58.
Detwiler, L. A.,, and M. Baylis. 2003. The epidemiology of scrapie. Rev. Sci. Tech. 22: 121– 143.
59.
Dietz, K.,, G. Raddatz,, J. Wallis,, N. Muller,, I. Zerr,, H. P. Duerr,, H. Lefevre,, E. Seifried, and, J. Lower. 2007. Blood transfusion and spread of variant Creutzfeldt-Jakob disease. Emerg. Infect. Dis. 13: 89– 96.
60.
Donnelly, C. A.,, N. M. Ferguson,, A. C. Ghani, and, R. M. Anderson. 2002. Implications of BSE infection screening data for the scale of the British BSE epidemic and current European infection levels. Proc. Biol. Sci. 269: 2179– 2190.
61.
Donnelly, C. A.,, N. M. Ferguson,, A. C. Ghani,, M. E. Woolhouse,, C. J. Watt, and, R. M. Anderson. 1997. The epidemiology of BSE in cattle herds in Great Britain. I. Epidemiological processes, demography of cattle and approaches to control by culling. Philos. Trans. R. Soc. Lond. B 352: 781– 801.
62.
Duffy, P.,, J. Wolf,, G. Collins,, A. G. DeVoe,, B. Streeten, and, D. Cowen. 1974. Possible person-to-person transmission of Creutzfeldt-Jakob disease. N. Engl. J. Med. 290: 692– 693. (Letter.)
63.
Esmonde, T. F.,, R. G. Will,, J. M. Slattery,, R. Knight,, R. Harries-Jones,, R. de Silva, and, W. B. Matthews. 1993. Creutzfeldt-Jakob disease and blood transfusion. Lancet 341: 205– 207.
64.
Federal Register. 2005. Agricultural Bioterrorism Protection Act of 2002; possession, use, and transfer of biological agents and toxins, final rule. Fed. Regist. 70: 13242– 13292. http://www.selectagents.gov/resources/APHISFinalRule.pdf. Accessed 16 March 2009.
65.
Fichet, G.,, E. Comoy,, C. Duval,, K. Antloga,, C. Dehen,, A. Charbonnier,, G. McDonnell,, P. Brown,, C. I. Lasmezas, and, J. P. Deslys. 2004. Novel methods for disinfection of prion-contaminated medical devices. Lancet 364: 521– 526.
66.
Flechsig, E.,, D. Shmerling,, I. Hegyi,, A. J. Raeber,, M. Fischer,, A. Cozzio,, C. von Mering,, A. Aguzzi, and, C. Weissmann. 2000. Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice. Neuron 27: 399– 408.
67.
Ford, M. J.,, L. J. Burton,, R. J. Morris, and, S. M. Hall. 2002. Selective expression of prion protein in peripheral tissues of the adult mouse. Neuroscience 113: 177– 192.
68.
Fraser, H.,, G. R. Pearson,, I. McConnell,, M. E. Bruce,, J. M. Wyatt, and, T. J. Gruffydd-Jones. 1994. Transmission of feline spongiform encephalopathy to mice. Vet. Rec. 134: 449.
69.
Gajdusek, D. C. 1977. Unconventional viruses and the origin and disappearance of kuru. Science 197: 943– 960.
70.
Gajdusek, D. C.,, C. J. Gibbs, and, M. Alpers. 1966. Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature 209: 794– 796.
71.
Gambetti, P.,, Z. Dong,, J. Yuan,, X. Xiao,, M. Zheng,, A. Alshekhlee,, R. Castellani,, M. Cohen,, M. A. Barria,, D. Gonzalez-Romero,, E. D. Belay,, L. B. Schonberger,, K. Marder,, C. Harris,, J. R. Burke,, T. Montine,, T. Wisniewski,, D. W. Dickson,, C. Soto,, C. M. Hulette,, J. A. Mastrianni,, Q. Kong, and, W. Q. Zou. 2008. A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63: 697– 708.
72.
Gambetti, P.,, P. Parchi, and, S. G. Chen. 2003. Hereditary Creutzfeldt-Jakob disease and fatal familial insomnia. Clin. Lab. Med. 23: 43– 64.
73.
Gibbs, C. J., Jr.,, D. M. Asher,, A. Kobrine,, H. L. Amyx,, M. P. Sulima, and, D. C. Gajdusek. 1994. Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery. J. Neurol. Neurosurg. Psychiatry 57: 757– 758.
74.
Gibbs, C. J., Jr.,, D. C. Gajdusek,, D. M. Asher,, M. P. Alpers,, E. Beck,, P. M. Daniel, and, W. B. Matthews. 1968. Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee. Science 161: 388– 389.
75.
Gigi, A.,, E. Vakil,, E. Kahana, and, U. Hadar. 2005. Presymptomatic signs in healthy CJD mutation carriers. Dement. Geriatr. Cogn. Disord. 19: 246– 255.
76.
Glatzel, M.,, E. Abela,, M. Maissen, and, A. Aguzzi. 2003. Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. N. Engl. J. Med. 349: 1812– 1820.
77.
Glatzel, M.,, K. Stoeck,, H. Seeger,, T. Luhrs, and, A. Aguzzi. 2005. Human prion diseases: molecular and clinical aspects. Arch. Neurol. 62: 545– 552.
78.
Gordon, W. S. 1946. Advances in veterinary research: Louping-ill tick-borne fever and scrapie. Vet. Res. 58: 516– 520.
79.
Goudsmit, J.,, C. H. Morrow,, D. M. Asher,, R. T. Yanagihara,, C. L. Masters,, C. J. Gibbs, Jr., and, D. C. Gajdusek. 1980. Evidence for and against the transmissibility of Alzheimer disease. Neurology 30: 945– 950.
80.
Grassi, J.,, S. Maillet,, S. Simon, and, N. Morel. 2008. Progress and limits of TSE diagnostic tools. Vet. Res. 39: 33.
81.
Groschup, M. H.,, and A. Buschmann. 2008. Rodent models for prion diseases. Vet. Res. 39: 32.
82.
Hadlow, W. J. 1959. Scrapie and kuru. Lancet 274: 289– 290.
83.
Harman, J. L.,, and C. J. Silva. 2009. Bovine spongiform encephalopathy. J. Am. Vet. Med. Assoc. 234: 59– 72.
84.
Harris, D. A. 2003. Trafficking, turnover and membrane topology of PrP. Br. Med. Bull. 66: 71– 85.
85.
Herzberg, L.,, B. N. Herzberg,, C. J. Gibbs, Jr.,, W. Sullivan,, H. Amyx, and, D. C. Gajdusek. 1974. Creutzfeldt-Jakob disease: hypothesis for high incidence in Libyan Jews in Israel. Science 186: 848. (Letter.)
86.
Hewitt, P. E.,, C. A. Llewelyn,, J. Mackenzie, and, R. G. Will. 2006. Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological Review study. Vox Sang. 91: 221– 230.
87.
Hill, A. F.,, R. J. Butterworth,, S. Joiner,, G. Jackson,, M. N. Rossor,, D. J. Thomas,, A. Frosh,, N. Tolley,, J. E. Bell,, M. Spencer,, A. King,, S. Al-Sarraj,, J. W. Ironside,, P. L. Lantos, and, J. Collinge. 1999. Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet 353: 183– 189.
88.
Hill, A. F.,, M. Desbruslais,, S. Joiner,, K. C. Sidle,, I. Gowland,, J. Collinge,, L. J. Doey, and, P. Lantos. 1997. The same prion strain causes vCJD and BSE. Nature 389: 448– 450, 526.
89.
Hilton, D. A.,, A. C. Ghani,, L. Conyers,, P. Edwards,, L. McCardle,, D. Ritchie,, M. Penney,, D. Hegazy, and, J. W. Ironside. 2004. Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J. Pathol. 203: 733– 739.
90.
Hsiao, K. K.,, D. Groth,, M. Scott,, S. L. Yang,, H. Serban,, D. Rapp,, D. Foster,, M. Torchia,, S. J. Dearmond, and, S. B. Prusiner. 1994. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc. Natl. Acad. Sci. USA 91: 9126– 9130.
91.
Hsiao, K. K.,, M. Scott,, D. Foster,, D. F. Groth,, S. J. DeArmond, and, S. B. Prusiner. 1990. Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 250: 1587– 1590.
92.
Ironside, J. W.,, M. T. Bishop,, K. Connolly,, D. Hegazy,, S. Lowrie,, M. Le Grice,, D. L. Ritchie,, L. M. McCardle, and, D. A. Hilton. 2006. Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ 332: 1186– 1188.
93.
Ironside, J. W.,, D. A. Hilton,, A. Ghani,, N. J. Johnston,, L. Conyers,, L. M. McCardle, and, D. Best. 2000. Retrospective study of prion-protein accumulation in tonsil and appendix tissues. Lancet 355: 1693– 1694.
94.
Ironside, J. W.,, K. Sutherland,, J. E. Bell,, L. McCardle,, C. Barrie,, K. Estebeiro,, M. Zeidler, and, R. G. Will. 1996. A new variant of Creutzfeldt-Jakob disease: neuropathological and clinical features. Cold Spring Harb. Symp. Quant. Biol. 61: 523– 530.
95.
Jones, M.,, A. Peden,, C. Prowse,, A. Groner,, J. Manson,, M. Turner,, J. Ironside,, I. Macgregor, and, M. Head. 2007. In vitro amplification and detection of variant Creutzfeldt-Jakob disease PrP(Sc). J. Pathol. 213: 21– 26.
96.
Kimberlin, R. H.,, S. Cole, and, C. A. Walker. 1987. Temporary and permanent modifications to a single strain of mouse scrapie on transmission to rats and hamsters. J. Gen. Virol. 68: 1875– 1881.
97.
King, S. 2008. French doctors on trial for manslaughter. Lancet 371: 637.
98.
Kirkwood, J. K.,, and A. A. Cunningham. 1994. Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles. Vet. Rec. 135: 296– 303.
99.
Klatzo, I.,, D. C. Gajdusek, and, V. Zigas. 1959. Pathology of Kuru. Lab. Invest. 8: 799– 847.
100.
Knight, R. 1998. The diagnosis of prion diseases. Parasitology 117(Suppl) : S3– S11.
101.
Kocisko, D. A.,, and B. Caughey. 2006. Searching for anti-prion compounds: cell-based high-throughput in vitro assays and animal testing strategies. Methods Enzymol. 412: 223– 234.
102.
Kong, Q.,, S. Huang,, W. Zou,, D. Vanegas,, M. Wang,, D. Wu,, J. Yuan,, M. Zheng,, H. Bai,, H. Deng,, K. Chen,, A. L. Jenny,, K. O’Rourke,, E. D. Belay,, L. B. Schonberger,, R. B. Petersen,, M. S. Sy,, S. G. Chen, and, P. Gambetti. 2005. Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. J. Neurosci. 25: 7944– 7949.
103.
Kong, Q.,, W. K. Surewicz,, R. B. Petersen,, W. Q. Zou,, S. G. Chen,, P. Gambetti,, P. Parchi,, S. Capellari,, L. G. Goldfarb,, P. Montagna,, E. Lugaresi,, P. Picardo, and, B. Ghetti. 2004. Inherited prion diseases, p. 673–775. In S. B. Prusiner (ed.), Prion Biology and Diseases, 2nd ed. Cold Spring Harbor Laboratory Press, New York, NY.
104.
Kovacs, G. G.,, M. Puopolo,, A. Ladogana,, M. Pocchiari,, H. Budka,, C. van Duijn,, S. J. Collins,, A. Boyd,, A. Giulivi,, M. Coulthart,, N. Delasnerie-Laupretre,, J. P. Brandel,, I. Zerr,, H. A. Kretzschmar,, J. de Pedro-Cuesta,, M. Calero-Lara,, M. Glatzel,, A. Aguzzi,, M. Bishop,, R. Knight,, G. Belay,, R. Will, and, E. Mitrova. 2005. Genetic prion disease: the EUROCJD experience. Hum. Genet. 118: 166– 174.
105.
Krasnianski, A.,, B. Meissner,, U. Heinemann, and, I. Zerr. 2004. Clinical findings and diagnostic tests in Creutzfeldt-Jakob disease and variant Creutzfeldt-Jakob disease. Folia Neuropathol. 42( Suppl. B) : 24– 38.
106.
Kuczius, T.,, R. Koch,, K. Keyvani,, H. Karch,, J. Grassi, and, M. H. Groschup. 2007. Regional and phenotype heterogeneity of cellular prion proteins in the human brain. Eur. J. Neurosci. 25: 2649– 2655.
107.
Ladogana, A.,, M. Puopolo,, E. A. Croes,, H. Budka,, C. Jarius,, S. Collins,, G. M. Klug,, T. Sutcliffe,, A. Giulivi,, A. Alperovitch,, N. Delasnerie-Laupretre,, J. P. Brandel,, S. Poser,, H. Kretzschmar,, I. Rietveld,, E. Mitrova,, P. Cuesta Jde,, P. Martinez-Martin,, M. Glatzel,, A. Aguzzi,, R. Knight,, H. Ward,, M. Pocchiari,, C. M. van Duijn,, R. G. Will, and, I. Zerr. 2005. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology 64: 1586– 1591.
108.
Lasmezas, C. I.,, J. P. Deslys,, R. Demaimay,, K. T. Adjou,, F. Lamoury,, D. Dormont,, O. Robain,, J. Ironside, and, J. J. Hauw. 1996. BSE transmission to macaques. Nature 381: 743– 744.
109.
Lewis, V.,, G. M. Klug,, A. F. Hill, and, S. J. Collins. 2008. Molecular typing of PrP(res) in human sporadic CJD brain tissue. Methods Mol. Biol. 459: 241– 247.
110.
Liberski, P. P.,, and P. Brown. 2007. Kuru—fifty years later. Neurol. Neurochir. Pol. 41: 548– 556.
111.
Liberski, P. P.,, and H. Budka. 2004. Gerstmann-Straussler-Scheinker disease. I. Human diseases. Folia Neuropathol. 42( Suppl. B) : 120– 140.
112.
Lipp, H. P.,, M. Stagliar-Bozicevic,, M. Fischer, and, D. P. Wolfer. 1998. A 2-year longitudinal study of swimming navigation in mice devoid of the prion protein: no evidence for neurological anomalies or spatial learning impairments. Behav. Brain Res. 95: 47– 54.
113.
Mallucci, G.,, A. Dickinson,, J. Linehan,, P. C. Klohn,, S. Brandner, and, J. Collinge. 2003. Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science 302: 871– 874.
114.
Mallucci, G. R.,, M. D. White,, M. Farmer,, A. Dickinson,, H. Khatun,, A. D. Powell,, S. Brandner,, J. G. Jefferys, and, J. Collinge. 2007. Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron 53: 325– 335.
115.
Marsh, R. F.,, and W. J. Hadlow. 1992. Transmissible mink encephalopathy. Rev. Sci. Tech. 11: 539– 550.
116.
Matthews, W. B.,, and R. G. Will. 1981. Creutzfeldt-Jakob disease in a lifelong vegetarian. Lancet ii: 937.
117.
Mawhinney, S.,, W. J. Pape,, J. E. Forster,, C. A. Anderson,, P. Bosque, and, M. W. Miller. 2006. Human prion disease and relative risk associated with chronic wasting disease. Emerg. Infect. Dis. 12: 1527– 1535.
118.
Mead, S. 2006. Prion disease genetics. Eur. J. Hum. Genet. 14: 273– 281.
119.
Mead, S.,, S. Joiner,, M. Desbruslais,, J. A. Beck,, M. O’Donoghue,, P. Lantos,, J. D. Wadsworth, and, J. Collinge. 2007. Creutzfeldt-Jakob disease, prion protein gene codon 129VV, and a novel PrPSc type in a young British woman. Arch. Neurol. 64: 1780– 1784.
120.
Meiner, Z.,, R. Gabizon, and, S. B. Prusiner. 1997. Familial Creutzfeldt-Jakob disease. Codon 200 prion disease in Libyan Jews. Medicine (Baltimore) 76: 227– 237.
121.
Meyer-Luehmann, M.,, J. Coomaraswamy,, T. Bolmont,, S. Kaeser,, C. Schaefer,, E. Kilger,, A. Neuenschwander,, D. Abramowski,, P. Frey,, A. L. Jaton,, J. M. Vigouret,, P. Paganetti,, D. M. Walsh,, P. M. Mathews,, J. Ghiso,, M. Staufenbiel,, L. C. Walker, and, M. Jucker. 2006. Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science 313: 1781– 1784.
122.
Montagna, P.,, P. Gambetti,, P. Cortelli, and, E. Lugaresi. 2003. Familial and sporadic fatal insomnia. Lancet Neurol. 2: 167– 176.
123.
Muller-Schiffmann, A.,, and C. Korth. 2008. Vaccine approaches to prevent and treat prion infection: progress and challenges. BioDrugs 22: 45– 52.
124.
Murray, K.,, D. L. Ritchie,, M. Bruce,, C. A. Young,, M. Doran,, J. W. Ironside, and, R. G. Will. 2008. Sporadic creutzfeldt-jakob disease in two adolescents. J. Neurol. Neurosurg. Psychiatry 79: 14– 18.
125.
Nathanson, N.,, J. Wilesmith, and, C. Griot. 1997. Bovine spongiform encephalopathy (BSE): causes and consequences of a common source epidemic. Am. J. Epidemiol. 145: 959– 969.
126.
National Creutzfeldt-Jakob Disease Surveillance Unit. 2008. Variant Creuzfeldt-Jakob disease current data (February 2009). National CJD Surveillance Unit, Edinburgh, Scotland. http://www.cjd.ed.ac.uk/vcjdworld.htm. Accessed 16 March 2009.
127.
Reference deleted.
128.
Neuendorf, E.,, A. Weber,, A. Saalmueller,, H. Schatzl,, K. Reifenberg,, E. Pfaff, and, M. H. Groschup. 2004. Glycosylation deficiency at either one of the two glycan attachment sites of cellular prion protein preserves susceptibility to bovine spongiform encephalopathy and scrapie infections. J. Biol. Chem. 279: 53306– 53316.
129.
NIH. Appendix B-III-D, Risk group 3 (RG3) - viruses and prions. In NIH Guidelines for Research Involving Recombinant DNA Molecules. National Institutes of Health, Bethesda, MD. http://www4.od.nih.gov/oba/rac/guidelines_02/NIH_Guidelines_Apr_02.htm. Accessed 16 March 2009.
130.
Obici, L.,, V. Perfetti,, G. Palladini,, R. Moratti, and, G. Merlini. 2005. Clinical aspects of systemic amyloid diseases. Biochim. Biophys. Acta 1753: 11– 22.
131.
Oesch, B.,, D. Westaway,, M. Walchli,, M. P. McKinley,, S. B. Kent,, R. Aebersold,, R. A. Barry,, P. Tempst,, D. B. Teplow,, L. E. Hood,, S. B. Prusiner, and, C. Weissmann. 1985. A cellular gene encodes scrapie PrP 27-30 protein. Cell 40: 735– 746.
132.
Onisko, B. C.,, C. J. Silva,, I. Dynin,, M. Erickson,, W. H. Vensel,, R. Hnasko,, J. R. Requena, and, J. M. Carter. 2007. Sensitive, preclinical detection of prions in brain by nano-spray liquid chromatography/tandem mass spectrometry. Rapid Commun. Mass Spectrom. 21: 4023– 4026.
133.
Palmer, M. S.,, A. J. Dryden,, J. T. Hughes, and, J. Collinge. 1991. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 352: 340– 342.
134.
Parry, H. B. 1962. Scrapie: a transmissible and hereditary disease of sheep. Heredity 17: 75– 105.
135.
Pattison, I. H. 1965. Experiments with scrapie with special reference to the nature of the agent and the pathology of the disease, p. 249–257. In C. J. Gajdusek,, C. J. Gibbs, and, M. P. Alpers (eds.), Slow, Latent, and Temperate Virus Infections. U.S. Government Printing Office, Washington, DC.
136.
Pattison, I. H. 1965. Resistance of the scrapie agent to formalin. J. Comp. Pathol. 75: 159– 164.
137.
Peden, A. H.,, M. W. Head,, D. L. Ritchie,, J. E. Bell, and, J. W. Ironside. 2004. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 364: 527– 529.
138.
Peden, A. H.,, D. L. Ritchie,, M. W. Head, and, J. W. Ironside. 2006. Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease. Am. J. Pathol. 168: 927– 935.
139.
Peet, R. L.,, and J. M. Curran. 1992. Spongiform encephalopathy in an imported cheetah ( Acinonyx jubatus). Aust. Vet. J. 69: 171.
140.
Peretz, D.,, S. Supattapone,, K. Giles,, J. Vergara,, Y. Freyman,, P. Lessard,, J. G. Safar,, D. V. Glidden,, C. McCulloch,, H. O. Nguyen,, M. Scott,, S. J. Dearmond, and, S. B. Prusiner. 2006. Inactivation of prions by acidic sodium dodecyl sulfate. J. Virol. 80: 322– 331.
141.
Piccardo, P.,, J. C. Manson,, D. King,, B. Ghetti, and, R. M. Barron. 2007. Accumulation of prion protein in the brain that is not associated with transmissible disease. Proc. Natl. Acad. Sci. USA 104: 4712– 4717.
142.
Prusiner, S. B. 2004. An introduction to prion biology and diseases, p. 1–87. In S. B. Prusiner (ed.), Prion Biology and Diseases, 2nd ed. Cold Spring Harbor Laboratory Press, Cold Spring Harbor, New York, NY.
143.
Prusiner, S. B. 1982. Novel proteinaceous infectious particles cause scrapie. Science 216: 136– 144.
144.
Prusiner, S. B. 1998. Prions. Proc. Natl. Acad. Sci. USA 95: 13363– 13383.
145.
Prusiner, S. B. 1984. Prions: novel infectious pathogens. Adv. Virus. Res. 29: 1– 56.
146.
Prusiner, S. B.,, M. Scott,, D. Foster,, K. M. Pan,, D. Groth,, C. Mirenda,, M. Torchia,, S. L. Yang,, D. Serban,, G. A. Carlson,, P. C. Hoppe,, D. Westaway, and, S. J. DeArmond. 1990. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell 63: 673– 686.
147.
Race, R. E.,, and G. J. Raymond. 2004. Inactivation of transmissible spongiform encephalopathy (prion) agents by environ LpH. J. Virol. 78: 2164– 2165.
148.
Richt, J. A.,, P. Kasinathan,, A. N. Hamir,, J. Castilla,, T. Sathiyaseelan,, F. Vargas,, J. Sathiyaseelan,, H. Wu,, H. Matsushita,, J. Koster,, S. Kato,, I. Ishida,, C. Soto,, J. M. Robl, and, Y. Kuroiwa. 2007. Production of cattle lacking prion protein. Nat. Biotechnol. 25: 132– 138.
149.
Riesner, D. 2003. Biochemistry and structure of PrP(C) and PrP(Sc). Br. Med. Bull. 66: 21– 33.
150.
Rudd, P. M.,, T. Endo,, C. Colominas,, D. Groth,, S. F. Wheeler,, D. J. Harvey,, M. R. Wormald,, H. Serban,, S. B. Prusiner,, A. Kobata, and, R. A. Dwek. 1999. Glycosylation differences between the normal and pathogenic prion protein isoforms. Proc. Natl. Acad. Sci. USA 96: 13044– 13049.
151.
Saborio, G. P.,, B. Permanne, and, C. Soto. 2001. Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature 411: 810– 813.
152.
Safar, J.,, H. Wille,, V. Itri,, D. Groth,, H. Serban,, M. Torchia,, F. E. Cohen, and, S. B. Prusiner. 1998. Eight prion strains have PrP(Sc) molecules with different conformations. Nat. Med. 4: 1157– 1165.
153.
Scott, M.,, D. Foster,, C. Mirenda,, D. Serban,, F. Coufal,, M. Walchli,, M. Torchia,, D. Groth,, G. Carlson,, S. J. DeArmond,, D. Westaway, and, S. B. Prusiner. 1989. Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell 59: 847– 857.
154.
Shiga, Y.,, K. Satoh,, T. Kitamoto,, S. Kanno,, I. Nakashima,, S. Sato,, K. Fujihara,, H. Takata,, K. Nobukuni,, S. Kuroda,, H. Takano,, Y. Umeda,, H. Konno,, K. Nagasato,, A. Satoh,, Y. Matsuda,, M. Hidaka,, H. Takahashi,, Y. Sano,, K. Kim,, T. Konishi,, K. Doh-ura,, T. Sato,, K. Sasaki,, Y. Nakamura,, M. Yamada,, H. Mizusawa, and, Y. Itoyama. 2007. Two different clinical phenotypes of Creutzfeldt-Jakob disease with a M232R substitution. J. Neurol. 254: 1509– 1517.
155.
Si, K.,, S. Lindquist, and, E. R. Kandel. 2003. A neuronal isoform of the aplysia CPEB has prion-like properties. Cell 115: 879– 891.
156.
Somerville, R. A.,, A. Chong,, O. U. Mulqueen,, C. R. Birkett,, S. C. Wood, and, J. Hope. 1997. Biochemical typing of scrapie strains. Nature 386: 564.
157.
Stahl, N.,, M. A. Baldwin,, R. Hecker,, K. M. Pan,, A. L. Burlingame, and, S. B. Prusiner. 1992. Glycosylinositol phospholipid anchors of the scrapie and cellular prion proteins contain sialic acid. Biochemistry 31: 5043– 5053.
158.
Stahl, N.,, M. Baldwin,, D. B. Teplow,, C. E. Hood,, R. Beavis,, J. B. Chait,, B. Bibson,, A. L. Bulingame, and, S. B. Prusiner. 1992. Cataloging post-translational modifications of the scrapie prion protein by mass spectrometry, p. 361–379. In S. B. Prusiner,, J. Collinge,, J. Powell, and, B. Anderton (eds), Prion Diseases of Humans and Animals. Ellis Horwood, New York, NY.
159.
Stewart, L. A.,, L. H. Rydzewska,, G. F. Keogh, and, R. S. Knight. 2008. Systematic review of therapeutic interventions in human prion disease. Neurology 70: 1272– 1281.
160.
Strumbo, B.,, S. Ronchi,, L. C. Bolis, and, T. Simonic. 2001. Molecular cloning of the cDNA coding for Xenopus laevis prion protein. FEBS Lett. 508: 170– 174.
161.
Supattapone, S.,, P. Bosque,, T. Muramoto,, H. Wille,, C. Aagaard,, D. Peretz,, H. O. Nguyen,, C. Heinrich,, M. Torchia,, J. Safar,, F. E. Cohen,, S. J. DeArmond,, S. B. Prusiner, and, M. Scott. 1999. Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic mice. Cell 96: 869– 878.
162.
Supattapone, S.,, E. Bouzamondo,, H. L. Ball,, H. Wille,, H. O. Nguyen,, F. E. Cohen,, S. J. DeArmond,, S. B. Prusiner, and, M. Scott. 2001. A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice. Mol. Cell. Biol. 21: 2608– 2616.
163.
Supervie, V.,, and D. Costagliola. 2004. The unrecognised French BSE epidemic. Vet. Res. 35: 349– 362.
164.
Taylor, D. M. 2000. Inactivation of transmissible degenerative encephalopathy agents: a review. Vet. J. 159: 10– 17.
165.
Taylor, D. M. 2004. Resistance of transmissible spongiform encephalopathy agents to decontamination. Contrib. Microbiol. 11: 136– 145.
166.
Taylor, D. M.,, A. G. Dickinson,, H. Fraser,, P. A. Robertson,, P. R. Salacinski, and, P. J. Lowry. 1985. Preparation of growth hormone free from contamination with unconventional slow viruses. Lancet ii: 260– 262.
167.
Tennent, G. A.,, M. W. Head,, M. Bishop,, P. N. Hawkins,, R. G. Will,, R. Knight,, A. H. Peden,, L. M. McCardle,, J. W. Ironside, and, M. B. Pepys. 2007. Disease-associated prion protein is not detectable in human systemic amyloid deposits. J. Pathol. 213: 376– 383.
168.
Todd, N. V.,, J. Morrow,, K. Doh-ura,, S. Dealler,, S. O’Hare,, P. Farling,, M. Duddy, and, N. G. Rainov. 2005. Cerebroventricular infusion of pentosan polysulphate in human variant Creutzfeldt-Jakob disease. J. Infect. 50: 394– 396.
169.
Trevitt, C. R.,, and J. Collinge. 2006. A systematic review of prion therapeutics in experimental models. Brain 129: 2241– 2265.
170.
Trifilo, M. J.,, M. Sanchez-Alavez,, L. Solforosi,, J. Bernard-Trifilo,, S. Kunz,, D. McGavern, and, M. B. Oldstone. 2008. Scrapie-induced defects in learning and memory of transgenic mice expressing anchorless prion protein are associated with alterations in the GABAergic pathway. J. Virol. 82: 9890– 9899.
171.
Tuzi, N. L.,, E. Cancellotti,, H. Baybutt,, L. Blackford,, B. Bradford,, C. Plinston,, A. Coghill,, P. Hart,, P. Piccardo,, R. M. Barron, and, J. C. Manson. 2008. Host PrP glycosylation: a major factor determining the outcome of prion infection. PLoS Biol. 6: e100.
172.
Wadsworth, J. D.,, E. A. Asante,, M. Desbruslais,, J. M. Linehan,, S. Joiner,, I. Gowland,, J. Welch,, L. Stone,, S. E. Lloyd,, A. F. Hill,, S. Brandner, and, J. Collinge. 2004. Human prion protein with valine 129 prevents expression of variant CJD phenotype. Science 306: 1793– 1796.
173.
Wadsworth, J. D.,, S. Joiner,, A. F. Hill,, T. A. Campbell,, M. Desbruslais,, P. J. Luthert, and, J. Collinge. 2001. Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet 358: 171– 180.
174.
Wadsworth, J. D.,, C. Powell,, J. A. Beck,, S. Joiner,, J. M. Linehan,, S. Brandner,, S. Mead, and, J. Collinge. 2008. Molecular diagnosis of human prion disease. Methods Mol. Biol. 459: 197– 227.
175.
Weissmann, C.,, and E. Flechsig. 2003. PrP knock-out and PrP transgenic mice in prion research. Br. Med. Bull. 66: 43– 60.
176.
Wells, G. A.,, A. C. Scott,, C. T. Johnson,, R. F. Gunning,, R. D. Hancock,, M. Jeffrey,, M. Dawson, and, R. Bradley. 1987. A novel progressive spongiform encephalopathy in cattle. Vet. Rec. 121: 419– 420.
177.
Whittle, I. R.,, R. S. Knight, and, R. G. Will. 2006. Unsuccessful intraventricular pentosan polysulphate treatment of variant Creutzfeldt-Jakob disease. Acta Neurochir. (Wien) 148: 677– 679.
178.
Wickner, R. B.,, H. K. Edskes,, B. T. Roberts,, U. Baxa,, M. M. Pierce,, E. D. Ross, and, A. Brachmann. 2004. Prions: proteins as genes and infectious entities. Genes Dev. 18: 470– 485.
179.
Wilesmith, J. W.,, G. A. Wells,, M. P. Cranwell, and, J. B. Ryan. 1988. Bovine spongiform encephalopathy: epidemiological studies. Vet. Rec. 123: 638– 644.
180.
Will, R. G.,, A. Alperovitch,, S. Poser,, M. Pocchiari,, A. Hofman,, E. Mitrova,, R. de Silva,, M. D’Alessandro,, N. Delasnerie-Laupretre,, I. Zerr, and, C. van Duijn. 1998. Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993-1995. EU Collaborative Study Group for CJD. Ann. Neurol. 43: 763– 767.
181.
Will, R. G.,, J. W. Ironside,, M. Zeidler,, S. N. Cousens,, K. Estibeiro,, A. Alperovitch,, S. Poser,, M. Pocchiari,, A. Hofman, and, P. G. Smith. 1996. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347: 921– 925.
182.
Williams, E. S. 2005. Chronic wasting disease. Vet. Pathol. 42: 530– 549.
183.
Wilson, K.,, C. Code, and, M. N. Ricketts. 2000. Risk of acquiring Creutzfeldt-Jakob disease from blood transfusions: systematic review of case-control studies. BMJ 321: 17– 19.
184.
Wopfner, F.,, G. Weidenhofer,, R. Schneider,, A. von Brunn,, S. Gilch,, T. F. Schwarz,, T. Werner, and, H. M. Schatzl. 1999. Analysis of 27 mammalian and 9 avian PrPs reveals high conservation of flexible regions of the prion protein. J. Mol. Biol. 289: 1163– 1178.
185.
World Health Organization. 1998. Global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies: report of a WHO consultation, Geneva, Switzerland, 9–11 February 1998. Report no. WHO/EMC/ZDI/98.9 World Health Organization, Geneva, Switzerland. http://www.who.int/csr/resources/publications/bse/whoemczdi989.pdf. Accessed 16 March 2009.
186.
World Health Organization. 1999. WHO infection control guidelines for transmissible spongiform encephalopathies. Report of a WHO consultation, Geneva, Switzerland, 23-26 March 1999. Report no. WHO/CDS/CSR/APH/2000/3. World Health Organization, Geneva, Switzerland. http://www.who.int/csr/resources/publications/bse/whocdscsraph2003.pdf. Accessed 16 March 2009.
187.
World Health Organization. 2001. The revision of the surveillance case definition for variant Creutzfeldt-Jakob Disease (vCJD). Report of a WHO consultation, Edinburgh, United Kingdom 17 May 2001. Report no. WHO/CDS/CSR/EPH/2001.5. World Health Organization, Geneva, Switzerland. http://www.who.int/csr/resources/publications/bse/whocdscsreph20015.pdf. Accessed 16 March 2009.
188.
World Organisation for Animal Health (OIE). 2008. Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide. July 24, 2008. World Organisation for Animal Health, Paris, France. http://www.oie.int/eng/info/en_esbmonde.htm. Accessed 16 March 2009.
189.
Wyatt, J. M.,, G. R. Pearson,, T. N. Smerdon,, T. J. Gruffydd-Jones,, G. A. Wells, and, J. W. Wilesmith. 1991. Naturally occurring scrapie-like spongiform encephalopathy in five domestic cats. Vet. Rec. 129: 233– 236.
190.
Zerr, I.,, and S. Poser. 2002. Clinical diagnosis and differential diagnosis of CJD and vCJD. With special emphasis on laboratory tests. APMIS 110: 88– 98.
191.
Zhang, B.,, Y. Une,, X. Fu,, J. Yan,, F. Ge,, J. Yao,, J. Sawashita,, M. Mori,, H. Tomozawa,, F. Kametani, and, K. Higuchi. 2008. Fecal transmission of AA amyloidosis in the cheetah contributes to high incidence of disease. Proc. Natl. Acad. Sci. USA 105: 7263– 7268.
192.
Zomosa-Signoret, V.,, J. D. Arnaud,, P. Fontes,, M. T. Alvarez-Martinez, and, J. P. Liautard. 2008. Physiological role of the cellular prion protein. Vet. Res. 39: 9.
193.
Zou, S.,, C. T. Fang, and, L. B. Schonberger. 2008. Transfusion transmission of human prion diseases. Transfus. Med. Rev. 22: 58– 69.
Tables
Prion Diseases
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Citation:
Silva C.
2009.
Prion Diseases,
p 425-442.
In
Fratamico P, Smith J, Brogden K (ed),
Sequelae and Long-Term Consequences of Infectious Diseases.
ASM Press, Washington, DC.
doi: 10.1128/9781555815486.ch24
Table 1.
WHO case definition for CJD ( 182 )
Citation:
Silva C.
2009.
Prion Diseases,
p 425-442.
In
Fratamico P, Smith J, Brogden K (ed),
Sequelae and Long-Term Consequences of Infectious Diseases.
ASM Press, Washington, DC.
doi: 10.1128/9781555815486.ch24
Table 2.
WHO case definition for vCJD ( 183 )
Citation:
Silva C.
2009.
Prion Diseases,
p 425-442.
In
Fratamico P, Smith J, Brogden K (ed),
Sequelae and Long-Term Consequences of Infectious Diseases.
ASM Press, Washington, DC.
doi: 10.1128/9781555815486.ch24
/content/10.1128/9781555815486.ch24.ch24tab03
Table 3.