Animal prion diseases

Subcellular Biochemistry

Volumn 65, Issue , 2012, Pages 497-516

  Windl, Otto ^a   Dawson, Mike ^a  

^a VETERINARY LABORATORIES AGENCY   (United Kingdom)

Author keywords

Animal prion disease; Bovine spongiform encephalopathy (BSE); Rodent models; Scrapie

Indexed keywords

ANIMAL; BOVINE SPONGIFORM ENCEPHALOPATHY; BRAIN; CATTLE; DISEASE MODEL; GENETICS; GOAT; METABOLISM; MOUSE; PATHOLOGY; PRION; REVIEW; SCRAPIE; SHEEP; SPINAL CORD; TRANSGENIC MOUSE;

ANIMALS; BRAIN; CATTLE; DISEASE MODELS, ANIMAL; ENCEPHALOPATHY, BOVINE SPONGIFORM; GOATS; MICE; MICE, TRANSGENIC; PRIONS; SCRAPIE; SHEEP; SPINAL CORD;

EID: 84879801973     PISSN: 03060225     EISSN: None     Source Type: Book Series    
DOI: 10.1007/978-94-007-5416-4_18     Document Type: Article

References (88)

1. Andréoletti O, Lacroux C, Chabert A, Monnereau L, Tabouret G, Lantier F, Berthon P, Eychenne F, Lafond-Benestad S, Elsen J-M, Schelcher F (2002) PrPSc accumulation in placentas of ewes exposed to natural scrapie: influence of foetal PrP genotype and effect on ewe-to-lamb transmission. J GenVirol 83:2607-2616 (Expand+Journal of GeneralVirologyjgv.sgmjournals.org)
2. Atarashi R, Moore RA, Sim VL, HughsonAG, Dorward DW, Onwubiko HA, Priola SA, Caughey B (2007) Ultrasensitive detection of scrapie prion protein using seeded conversion of recombinant prion protein. Nat Methods 4:645-650
3. Balkema-Buschmann A, Fast C, Kaatz M, Eiden M, Ziegler U, McIntyre L, Keller M, Hills B, Groschup MH (2011) Pathogenesis of classical and atypical BSE in cattle. Prev Vet Med 102:112-117
4. Baron T, Bencsik A, BiacabeAG, Morignat E, Bessen RA (2007) Phenotypic similarity of transmissible mink encephalopathy in cattle and L-type bovine spongiform encephalopathy in a mouse model. Emerg Infect Dis 13:1887-1894
5. Benestad SL, Sarradin P, Thu B, Schönheit J, Tranulis MA, Bratberg B (2003) Cases of scrapie with unusual features in Norway and designation of a new type, Nor98. Vet Rec 153:202-208
6. Béringue V, Andréoletti O, Le Dur A, Essalmani R, Vilotte JL, Lacroux C, Reine F, Herzog L, Biacabé AG, Baron T, Caramelli M, Casalone C, Laude HA (2007) Bovine prion acquires an epidemic bovine spongiform encephalopathy strain-like phenotype on interspecies transmission. J Neurosci 27:6965-6971
7. BiacabeAG, Laplanche JL, Ryder S, Baron T (2004) Distinct molecular phenotypes in bovine prion diseases. EMBO Rep 5:110-114
8. Biasini E, Turnbaugh JA, Unterberger U, Harris DA (2012) Prion protein at the crossroads of physiology and disease. Trends Neurosci 35:92-103
9. Browning SR, Mason GL, Seward T, Green M, Eliason GA, Mathiason C, Miller MW, Williams ES, Hoover E, Telling GC (2004) Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP. J Virol 78:13345-13350
10. Bruce ME,Will R, Ironside J, McConnell I, Drummond D, Suttie A, McCardle L, Chree A, Hope J, Birkett C, Cousens S, Fraser H, Bostock C (1997) Transmissions to mice indicate that 'New Variant' CJD is caused by the BSE agent. Nature 389:498-501
11. Bruce ME, Boyle A, Cousens S, McConnell I, Foster J, Goldmann W, Fraser H (2002) Strain characterization of natural sheep scrapie and comparison with BSE. J Gen Virol 83:695-704
12. Budka H, Aguzzi A, Brown P, Brucher JM, Bugiani O, Collinge J, Diringer H, Gullotta F, Haltia M, Hauw JJ (1995) Tissue handling in suspected Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases). Brain Pathol 5:319-322
13. Büeler H, Aguzzi A, Sailer A, Greiner RA,Autenried P, Aguet M,Weissmann C (1993) Mice devoid of PrP are resistant to scrapie. Cell 73:1339-1347
14. Buschmann A, Gretzschel A, Biacabe AG, Schiebel K, Corona C, Hoffmann C, Eiden M, Baron T, Casalone C, Groschup MH (2006) Atypical BSE in Germany-proof of transmissibility and biochemical characterization. Vet Microbiol 117:103-116
15. Capobianco R, Casalone C, Suardi S, Mangieri M, Miccolo C, Limido L, Catania M, Rossi G, Di Fede G, Giaccone G, Bruzzone MG, Minati L, Corona C, Acutis P, Gelmetti D, Lombardi G, Groschup MH, Buschmann A, Zanusso G, Monaco S, Caramelli M, Tagliavini F (2007) Conversion of the BASE prion strain into the BSE strain: the origin of BSE? PLoS Pathol 3(3):e31
16. Casalone C, Zanusso G, Acutis P, Ferrari S, Capucci L, Tagliavini F, Monaco S, Caramelli M (2004) Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci USA 101:3065-3070
17. Collinge J, Sidle KCL, Meads J, Ironside JW, Hills AF (1996) Molecular analysis of prion strain variation and the aetiology of 'New Variant' CJD. Nature 383:685-690
18. Cosseddu GM, Nonno R, Vaccari G, Bucalossi C, Fernandez-Borges N, Di Bari MA, Castilla J, Agrimi U (2011) Ultra-efficient PrP(Sc) amplification highlights potentialities and pitfalls of PMCA technology. PLoS Pathol 7(11):e1002370
19. Dawson M, Wells GAH, Parker BNJ (1990) Preliminary evidence of the experimental transmissibility of bovine spongiform encephalopathy to cattle. Vet Rec 126:112-113
20. Dawson M, Moore RC, Bishop SC (2008) Progress and limits of PrP gene selection policy.Vet Res 39:25
21. Dickinson A, Stamp J, Renwick C, Renne J (1968) Some factors controlling the incidence of scrapie in cheviot sheep injected with a cheviot-passaged scrapie agent. J Comp Path 78:313-320
22. EFSA Panel on Biological Hazards (BIOHAZ) (2011) Joint scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans. EFSA J 9:1945
23. Eloit M, Adjou K, Coulpier M, Fontaine JJ, Hamel R, Lilin T, Messiaen S, Andreoletti O, Baron T, Bencsik A, BiacabeAG, BeringueV, Laude H, Le Dur A,Vilotte JL, Comoy E, Deslys JP, Grassi J, Simon S, Lantier F, Sarradin P (2005) BSE agent signatures in a goat. Vet Rec 156:523-524
24. Fediaevsky A, Maurella C, Nöremark M, Ingravalle F, Thorgeirsdottir S, Orge L, Poizat R, Hautaniemi M, Liam B, Calavas D, Ru G, Hopp P (2010) The prevalence of atypical scrapie in sheep from positive flocks is not higher than in the general sheep population in 11 European countries. BMC Vet Res 6:9
25. Foster JD, Hope J, Fraser H (1993) Transmission of bovine spongiform encephalopathy to sheep and goats. Vet Rec 133:339-341
26. Fraser H, McConnell I, Wells GAH, Dawson M (1988) Transmission of bovine spongiform encephalopathy to mice. Vet Rec 123:472
27. Gilch S, Chitoor N, Taguchi Y, Stuart M, Jewell JE, Schätzl HM (2011) Chronic wasting disease. Top Curr Chem 305:51-77
28. Götte DR, Benestad SL, Laude H, Zurbriggen A, Oevermann A, Seuberlich T (2011) Atypical scrapie isolates involve a uniform prion species with a complex molecular signature. PLoS One 6(11):e27510
29. Gonzalez L, Dagleish MP, Martin S, Dexter G, Steele P, Finlayson J, Jeffrey M (2008) Diagnosis of preclinical scrapie in live sheep by the immunohistochemical examination of rectal biopsies. Vet Rec 162:397-403
30. Gonzalez L, Martin S, Hawkins SAC, Goldmann W, Jeffrey M, Sisó S (2010a) Pathogenesis of natural goat scrapie: modulation by host PRNP genotype and effect of co-existent conditions. Vet Res 41:48
31. Gonzalez L, Sisó S, Monleón E, Casalone C, van Keulen LJ, Balkema-Buschmann A, Ortiz-Peláez A, Iulini B, Langeveld JP, Hoffmann C, Badiola JJ, Jeffrey M, Acín C (2010b) Variability in disease phenotypes within a single PRNP genotype suggests the existence of multiple natural sheep scrapie strains within Europe. J Gen Virol 91:2630-2641
32. Green R, Horrocks C, Wilkinson A, Hawkins SA, Ryder SJ (2005) Primary isolation of the bovine spongiform encephalopathy agent in mice: agent definition based on a review of 150 transmissions. J Comp Pathol 132:117-131
33. Hadlow W (2007) Portrait of transmissible mink encephalopathy. In: Hörnlimann B, Riesner D, Kretzschmar H (eds) Prions in humans and animals. de Gruyter, Berlin, pp. 265-270
34. Hadlow WJ, Kennedy RC, Race RE (1982) Natural infection of Suffolk sheep with scrapie virus. J Infect Dis 146:657-664
35. Hewicker-Trautwein M, Bradley R (2007) Portrait of transmissible feline spongiform encephalopathy. In: Hörnlimann B, Riesner D, Kretzschmar H (eds) Prions in humans and animals. de Gruyter, Berlin, pp. 271-273
36. Hope J, Reekie LJ, Hunter N, Multhaup G, Beyreuther K, White H, ScottAC, Stack MJ, Dawson M, WellsGAH (1988) Fibrils from brains of cows with newcattle disease contain scrapie-associated protein. Nature 336:390-392
37. Hörnlimann B, Bachmann J, Bradley R (2007a) Portrait of bovine spongiform encephalopathy in cattle and other ungulates. In: Hörnlimann B, Riesner D, Kretzschmar H (eds) Prions in humans and animals. de Gruyter, Berlin, pp 233-249
38. Hörnlimann B, Riesner D, Kretzschmar H, Will RG, MacDiarmid SC, Wells GAH, Alpers MP, (2007b) Historical introduction. In: Hörnlimann B, Riesner D, Kretzschmar H (eds) Prions in humans and animals. de Gruyter, Berlin, pp 3-27
39. Hunter N (2003) Scrapie and experimental BSE in sheep. Br Med Bull 66:171-183
40. Hunter N, Goldmann W, Foster JD, Cairns D, Smith G (1997) Natural scrapie and PrP genotype: case-control studies in British sheep. Vet Rec 141:137-140
41. Jacobs JG, Langeveld JP, Biacabe AG, Acutis PL, Polak MP, Gavier-Widen D, Buschmann A, Caramelli M, Casalone C, Mazza M, Groschup M, Erkens JH, Davidse A, van Zijderveld FG, Baron T (2007) Molecular discrimination of atypical bovine spongiform encephalopathy strains from a geographical region spanning a wide area in Europe. J Clin Microbiol 45:1821-1829
42. Jacobs JG, Sauer M, van Keulen LJM, TangY, Bossers A, Langeveld JPM (2011) Differentiation of ruminant transmissible spongiform encephalopathy isolate types, including bovine spongiform encephalopathy and CH1641 scrapie. J Gen Virol 92:222-232
43. Jeffrey M, Martin S, Thomson JR, Dingwall WS, Begara-McGorum I González (2001) Onset and distribution of tissue PrP accumulation in scrapie-affected suffolk sheep as demonstrated by sequential necropsies and tonsillar biopsies. J Comp Path 125:48-57
44. Jeffrey M, Martin S, González L, Foster J, Langeveld JP, van Zijderveld FG, Grassi J, Hunter N (2006) Immunohistochemical features of PrP(d) accumulation in natural and experimental goat transmissible spongiform encephalopathies. J Comp Path 134:171-181
45. Kim TY, Shon HJ, Joo YS, Mun UK, Kang KS, LeeYS (2005) Additional cases of chronic wasting disease in imported deer in Korea. J Vet Med Sci 67:753-759
46. Kirkwood JK, CunninghamAA (2007) Portrait of prion diseases in zoo animals. In: Hörnlimann B, Riesner D, Kretzschmar H (eds) Prions in humans and animals. de Gruyter, Berlin, pp 250-256
47. Kittelberger R, Chaplin MJ, Simmons MM, Ramirez-Villaescusa A, McIntyre L, MacDiarmid SC, Hannah MJ, Jenner J, Bueno R, Bayliss D, Black H, Pigott CJ, O'Keefe JS (2010) Atypical scrapie/Nor98 in a sheep from New Zealand. J Vet Diagn Invest 22:863-875
48. Klöhn PC, Stoltze L, Flechsig E, Enari M, Weissmann C (2003) A quantitative, highly sensitive cell-based infectivity assay for mouse scrapie prions. Proc NatlAcad SciUSA100:11666-11671
49. Konold T, Lee YH, Stack MJ, Horrocks C, Green RB, Chaplin M, Simmons MM, Hawkins SAC, Lockey R, Spiropoulos J,Wilesmith JW,Wells GAH (2006) Different prion disease phenotypes result from inoculation of cattle with two temporally separated sources of sheep scrapie from Great Britain. BMC Vet Res 2:31
50. Konold T, Moore SJ, Bellworthy SJ, Simmons HA (2008) Evidence of scrapie transmission via milk. BMC Vet Res 4:14
51. Le Dur A, Béringue V, Andréoletti O, Reine F, Läı TL, Baron T, Bratberg B, Vilotte JL, Sarradin P, Benestad SL, Laude H (2005) A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes. Proc Natl Acad Sci USA 102:16031-16036
52. Ligios C, CanceddaMG,CartaA, Santucciu C, Maestrale C, Demontis F, Saba M, Patta C, DeMartini JC, Aguzzi A, Sigurdson CJ (2011) Sheep with scrapie and mastitis transmit infectious prions through the milk. J Virol 85:1136-1139
53. Maddison BC, Baker CA, Terry LA, Bellworthy SJ, Thorne L, Rees HC, Gough KC (2010) Environmental sources of scrapie prions. J Virol 84:11560-11562
54. Mathiason CK, Powers JG, Dahmes SJ, Osborn DA, Miller KV,Warren RJ, Mason GL, Hays SA, Hayes-Klug J, Seelig DM,Wild MA,Wolfe LL, Spraker TR, MillerMW, Sigurdson CJ, Telling GC, Hoover EA (2006) Infectious prions in the saliva and blood of deer with chronic wasting disease. Science 314:133-136
55. Marsh RF (1979) On the origin of transmissible mink encephalopathy. In: Prusiner SB, HadlowWJ (eds) Slow transmissible diseases of the nervous system, vol. 1. Academic Press, NewYork, pp 451-460
56. Nagaoka K, Yoshioka M, Shimozaki N, Yamamura T, Murayama Y, Yokoyama T, Mohri S (2010) Sensitive detection of scrapie prion protein in soil. Biochem Biophys Res Commun 397:626-630
57. Neale MH, Mountjoy SJ, Edwards JC,Vilette D, Laude H,Windl O, Saunders GC (2010) Infection of cell lines with experimental and natural ovine scrapie agents. J Virol 84:2444-2452
58. Nussbaum R, Henderson W, Pattison I, Elcock N, Davies D (1975) The establishment of sheep flocks of predictable susceptibility to experimental scrapie. Res Vet Sci 18:49-58
59. Okada H, IwamaruY, Imamura M, Masujin K, MatsuuraY, ShimizuY, Kasai K, Mohri S,Yokoyama T, Czub S (2011) Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits. Vet Res 42:79
60. Opinion of the scientific panel on Biological Hazards (2005) Opinion of the scientific panel on biological hazards on the request from the european commission on classification of atypical Transmissible Spongiform Encephalopathy (TSE) cases in small ruminants. EFSA J 276:1-30
61. O'Rourke KI, Duncan JV, Logan JR, Anderson AK, Norden DK,Williams ES, Combs BA, Stobart RH, Moss GE, Sutton DL (2002) Active surveillance for scrapie by third eyelid biopsy and genetic susceptibility testing of flocks of sheep in wyoming. Clin Diagn Lab Immunol 9:966-971
62. Parry H (1979) Elimination of natural scrapie in sheep by sire genotype selection. Nature 277:127- 129
63. Papasavva-Stylianou P,Windl O, Saunders G, Mavrikiou P, Toumazos P, Kakoyiannis C (2011) PrP gene polymorphisms in cyprus goats and their association with resistance or susceptibility to natural scrapie. Vet J 187:245-250
64. Pattison I, Hoare M, Jebbett J,WatsonW (1972) Spread of scrapie to sheep and goats by oral dosing with foetal membranes from scrapie-affected sheep. Vet Rec 90:465-468
65. Prusiner SB (1998) Prions. Proc Natl Acad Sci USA 95:13363-13383
66. Rane NS, Chakrabarti O, Feigenbaum L, HegdeRS (2010) Signal sequence insufficiency contributes to neurodegeneration caused by transmembrane prion protein. J Cell Biol 188:515-526
67. Ryder SJ, Dexter G E, Heasman L, Warner R, Moore SJ (2009) Accumulation and dissemination of prion protein in experimental sheep scrapie in the natural host. BMC Vet Res 5:9
68. Saborio GP, Permanne B, Soto C (2001) Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature 411:810-813
69. Sander P, Hamann H, Drögemüller C, Kashkevich K, Schiebel K, LeebT (2005)Bovine prion protein gene (PRNP) promoter polymorphisms modulate PRNP expression and may be responsible for differences in bovine spongiform encephalopathy susceptibility. J Biol Chem 280:37408-37414
70. Scott M, Foster D, Mirenda C, Serban D, Coufal F, Wälchli M, Torchia M, Groth D, Carlson G, DeArmond SJ, Westaway D, Prusiner SB (1989) Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell 59:847-857
71. Simmons MM, Harris P, Jeffrey M, Meek SC, Blamire IWH, Wells GAH (1996) BSE in Great Britain: consistency of the neurohistopathological findings in two random annual samples of clinically suspect cases. Vet Rec 138:175-177
72. Simmons MM, Konold T, Simmons HA, Spencer YI, Lockey R, Spiropoulos J, Everitt S, Clifford D (2007) Experimental transmission of atypical scrapie to sheep. BMC Vet Res 3:20
73. Sisó S, González L, JeffreyM (2010) Neuroinvasion in prion diseases: the roles of ascending neural infection and blood dissemination. Interdiscip Perspect Infect Dis 2010:747892
74. Stack MJ, Moore SJ, Davis A, Webb PR, Bradshaw JM, Lee YH, Chaplin M, Focosi-Snyman R, Thurston L, Spencer YI, Hawkins SA, Arnold ME, Simmons MM, Wells GA (2011) Bovine spongiform encephalopathy: investigation of phenotypic variation among passive surveillance cases. J Comp Pathol 144:277-288
75. Stockman S (1913) Scrapie: an obscure disease of sheep. J Comp Path 26:317-327
76. Thorne L, Terry LA (2008) In vitro amplification of PrPSc derived from the brain and blood of sheep infected with scrapie. J Gen Virol 89:3177-3184
77. Vaccari G, Panagiotidis CH, Acin C, Peletto S, Barillet F, Acutis P, Bossers A, Langeveld J, Van Keulen L, Sklaviadis T, Badiola JJ, Andréoletti O, Groschup MH,Agrimi U, Foster J, Goldmann W(2009) State-of-the-art review of goat TSE in the European Union, with special emphasis on PRNP genetics and epidemiology. Vet Res 40:48
78. Vaccari G, Scavia G, Sala M, Cosseddu GM, Chiappini B, Conte M, Esposito E, Lorenzetti R, Perfetti G, Marconi P, Scholl F, Barbaro K, Bella A, Nonno R, Agrimi U (2011) Protective effect of the AT137RQ and ARQK176 PrP allele against classical scrapie in Sarda breed sheep. Vet Res 40:19
79. van Keulen LJ, Schreuder BE, Vromans ME, Langeveld JP, Smits MA (2000) Pathogenesis of natural scrapie in sheep. Arch Virol Suppl 16:57-71
80. Wells GAH, Scott AC, Johnson CT, Gunning RF, Hancock RD, Jeffrey M, Dawson M, Bradley R (1987) A novel progressive spongiform encephalopathy in cattle. Vet Rec 121:419-420
81. Wells GA, Hawkins SA, Green RB,AustinAR, Dexter I, SpencerYI, Chaplin MJ, Stack MJ, Dawson M (1998) Preliminary observations on the pathogenesis of experimental bovine spongiform encephalopathy (BSE): an update. Vet Rec 142:103-106
82. Wells GAH, Konold T, Arnold ME, Austin AR, Hawkins SAC, Stack M, Simmons MM, Lee YH, Gavier-Widén D, Dawson M, Wilesmith JW (2007) Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle. J Gen Virol 88:1363-1373
83. Wilesmith J,Wells G, Cranwell M, Ryan J (1988) Bovine spongiform encephalopathy: epidemiological studies. Vet Rec 123:638-644
84. Wilesmith J, Ryan J, Atkinson M (1991) Bovine spongiform encephalopathy: epidemiological studies on the origin. Vet Rec 128:199-203
85. Wilesmith JW, Hoinville LJ, Ryan JB, Sayers AR (1992) Bovine spongiform encephalopathy: aspects of the clinical picture and analyses of possible changes 1986-1990.Vet Rec 130:197-201
86. Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alpervitch A, Poser S, Pocchiari M, Hofman A, Smith PG (1996) A new variant of creutzfeldt-jakob disease in the UK. Lancet 347:921-925
87. Williams E, Young S (1980) Chronic wasting disease of captive mule deer: a spongiform encephalopathy. JWildl Dis 16:89-98
88. Windl O, Buchholz M, NeubauerA, Schulz-SchaefferW, Groschup M,Walter S,Arendt S, Neumann M, Voss AK, Kretzschmar HA (2005) Breaking an absolute species barrier: transgenic mice expressing the mink PrP gene are susceptible to transmissible mink encephalopathy. J Virol 79:14971-14975