General aspects of transmissible spongiform encephalopathies and hypotheses about the agents

References

• Aiken , JM and Marsh , RF . 1990 . The Microbiol Rev , 54 : 242 – 6 .
   PubMedGoogle Scholar
• Aiken , JM , Williamson , JL , Borchardt , LM and Marsh , RF . 1990 . Presence of mitochondrial D‐loop DNA in scrapie‐infected brain preparations enriched for the prion protein . J Virol , 64 : 3265 – 8 .
   PubMed Web of Science ®Google Scholar
• Aiken , JM , Williamson , JL and Marsh , RF . 1989 . Evidence of mitochondrial involvement in scrapie infection . J Virol , 63 : 1689 – 94 .
   Web of Science ®Google Scholar
• Alper , T , Cramp , WA , Haig , DA and Clarke , MC . 1967 . Does the agent of scrapie replicate without nucleic acid? . Nature , 214 : 764 – 7 .
   PubMed Web of Science ®Google Scholar
• Alper , T , Haig , DA and Clarke , MC . 1966 . The exceptionally small size of the scrapie agent . Biochem Biophys Res Commun , 22 : 278 – 84 .
   PubMed Web of Science ®Google Scholar
• Barry , RA , Kent , SBH McKinley , MP . 1986 . Scrapie and cellular prion proteins share polypeptide epitopes . J Inf Dis , 153 : 848 – 54 .
   PubMed Web of Science ®Google Scholar
• Barry , RA and Prusiner , SB . 1986 . Monoclonal antibodies to the cellular and scrapie prion protein . J Inf Dis , 154 : 518 – 21 .
   PubMed Web of Science ®Google Scholar
• Basler , K , Oesch , B Scott , M . 1986 . Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene . Cell , 46 : 417 – 28 .
   PubMed Web of Science ®Google Scholar
• Bellinger‐Kawahara , C , Diener , TO , McKinley , MP , Groth , DF , Smith , DR and Prusiner , SB . 1987 . Purified scrapie prions resist inactivation by procedures that hydrolyse, modify, or shear nucleic acids . Virol , 160 : 271 – 4 .
   PubMed Web of Science ®Google Scholar
• Bolton , DC and Bendheim , PE . 1991 . “ Purification of scrapie agents: how far have we come? ” . In Transmissible spongiform encephalopathies. Current topics Microbiol Immunol Edited by: Chesebro , B . 39 – 55 .
   Google Scholar
• Bolton , DC , McKinley , MP and Prusiner , SB . 1982 . Identification of a protein that purifies with the major scrapie prion . Science , 218 : 1309 – 11 .
   PubMed Web of Science ®Google Scholar
• Bolton , DC , Meyer , RK and Prusiner , SB . 1985 . Scrapic PrP 27–30 is a sialoglycoprotein . J Virol , 53 : 596 – 606 .
   PubMed Web of Science ®Google Scholar
• Brown , P , Liberski , PP , Wolff , A and Gajdusek , DC . 1990 . Resistance of scrapie infectivity to steam autoclaving after formaldehyde fixation and limited survival after ashing at 360°C; practical and theoretical implications . J Infect Dis , 161 : 467 – 72 .
   PubMed Web of Science ®Google Scholar
• Bruce , ME and Fraser , H . 1991 . Scrapie strain variation and implications. Chesebro B (ed). Transmissible spongiform encephalopathies . Current topics Microbiol Immunol , 172 : 125 – 38 .
   PubMedGoogle Scholar
• Büeler , H , Fischer , M. Lang , Y . 1992 . Normal behaviour of mice lacking the neuronal cell‐surface PrP protein . Nature , 356 : 577 – 82 .
   PubMed Web of Science ®Google Scholar
• Carlson , GA , Kingsbury , DT Goodman , PA . 1986 . Linkage of prion protein and scrapie incubation time genes . Cell , 46 : 503 – 11 .
   PubMed Web of Science ®Google Scholar
• Carlson , GA , Westaway , D , DeArmond , SJ , Peterson‐Torchia , M and Prusiner , SB . 1989 . Primary structure of prion protein may modify scrapie isolate properties . Proc Natl Acad Sci USA , 86 : 7475 – 9 .
   PubMed Web of Science ®Google Scholar
• Carp , RI , Kascsak , RJ , Wisniewski , HM , Merz , PA , Rubinstein , R , Bendheim , P and Bolton , D . 1989 . The nature of the unconventional slow infection agents remains a puzzle . Alzheimer Disc Assoc Disorders , 3 : 79 – 99 .
   PubMedGoogle Scholar
• Carp , RI , Merz , PA , Kascsak , RJ , Merz , GS and Wisniewski , HM . 1985 . Nature of the scrapie agent: current facts and hypotheses . J gen Virol , 66 : 1357 – 68 .
   PubMed Web of Science ®Google Scholar
• Caughy , B , Race , RE and Chesebro , B . 1988 . Detection of prion protein mRNA in normal and scrapie‐infected tissues and cell lines . J Gen Virol , 69 : 711 – 6 .
   PubMed Web of Science ®Google Scholar
• Caughy , B and Raymond , GJ . 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 – 23 .
   PubMed Web of Science ®Google Scholar
• Chesebro , B . 1992 . PrP and the scrapie agent . Nature , 356 : 560
   PubMed Web of Science ®Google Scholar
• Chesebro , B , Race , R Wehrly , K . 1985 . Identification of a scrapie prion protein‐specific mRNA in scrapie‐infected and uninfected brain . Nature , 315 : 331 – 3 .
   PubMed Web of Science ®Google Scholar
• Dickinson , AG , Fraser , H and Ter Meulen , V . 1977 . “ Scrapie pathogenesis in inbred mice: an assessment of host control and response involving many strains of agent ” . In Slow virus infections of the central nervous system , Edited by: Katz , M . 3 – 16 . Berlin Heidelberg New York : Springer .
   Google Scholar
• Dickinson , AG and Outram , G . 1979 . “ The scrapie replication‐site hypothesis and its implications for pathogenesis ” . In Slow transmissible diseases of the nervous system , Edited by: Prusiner , SB and Hadlow , WJ . Vol. 2 , 13 – 31 . Academic Press .
   Google Scholar
• Dickinson , AG and Outram , G . Genetic aspects of unconventional virus infections: the basis of the virino hypothesis . Novel Infectious agents and the Central Nervous System. Ciba Found. Symp. 135 . Edited by: Bock , G and Marsh , J . pp. 63 UK : John Wiley & Sons .
   Google Scholar
• Dickinson , AG and Taylor , DM . 1978 . Resistance of scrapie agent to decontamination . N Eng J Med , 229 : 1413 – 4 .
   Google Scholar
• Diener , TO . 1972 . Is the scrapie agent a viroid? . Nature , 235 : 218 – 9 .
   Web of Science ®Google Scholar
• Diener , TO . 1987 . PrP and the nature of the scrapie agent . Cell , 49 : 719 – 21 .
   PubMed Web of Science ®Google Scholar
• Diener , TO , McKinley , MP and Prusiner , SB . 1982 . Viroids and prions . Proc Natl Acad Sci , 79 : 5220 – 4 .
   PubMed Web of Science ®Google Scholar
• Diringer , H . 1991 . Transmissible spongiform encephalopathies; virus‐induced amyloidoses of the central nervous system . Eur J Epidemiol , 7 : 562 – 6 .
   PubMed Web of Science ®Google Scholar
• Diringer , H . 1992 . “ The Virus Hypothesis ” . In Procs Int Meeting on Transmiss Spongif Encephalopathies Heidelberg in press
   Google Scholar
• Diringer , H , Gelderblom , H , Hilmert , H , Özel , M , Edelblut , C and Kimberlin , RH . 1983 . Scrapie infectivity, fibrils and low molecular weight protein . Nature , 306 : 476 – 8 .
   PubMed Web of Science ®Google Scholar
• Gajdusek , DC . 1988 . Transmissible and non‐tranmissible amyloidoses: autocatalytic post‐translational conversion of host‐precursor proteins to beta‐pleated sheet configurations . J Neuroimmunol , 20 : 95 – 110 .
   PubMed Web of Science ®Google Scholar
• Gibbs , CJ Jr , Gajdusek , DC and Amyx , H . 1979 . “ Strain variation in the viruses of Creutzfeldt‐Jakob disease and kuru ” . In Slow transmissible diseases of the nervous system , Edited by: Prusiner , SB and Hadlow , WJ . 87 – 110 . Academic Press .
   Google Scholar
• Goldmann , W , Hunter , N , Benson , G , Foster , JD and Hope , J . 1991 . Different scrapie‐associated fibril proteins (PrP) are encoded by lines of sheep selected for different alleles of the Sip gene . J gen Virol , 72 : 2411 – 7 .
   PubMed Web of Science ®Google Scholar
• Goldmann , W , Hunter , N , Foster , JD , Salbaum , JM , Beyreuther , K and Hope , J . 1990 . Two alleles of a neural protein gene linked to scrapie in sheep . Proc Natl Acad Sci USA , 87 : 2476 – 80 .
   PubMed Web of Science ®Google Scholar
• Goldmann , W , Hunter , N , Martin , T , Dawson , M and Hope , J . 1991 . Different forms of the bovine PrP gene have five or six copies of a short, G‐C‐rich element within the protein‐coding exon . J gen Virol , 72 : 201 – 4 .
   PubMed Web of Science ®Google Scholar
• Hardy , J . 1991 . Prion dimers: a deadly duo? . TINS , 14 : 423 – 4 .
   PubMed Web of Science ®Google Scholar
• Harris , DA , Falls , DL , Johnson , FA and Fischbach , GD . 1991 . A prion‐like protein from chicken brain copurifies with an acetyl‐choline receptor‐inducing activity . Proc. Natl Acad Sci USA , 88 : 764 – 8 .
   Web of Science ®Google Scholar
• Hope , J , Morton , LDJ , Farquhar , CF , Multhaup , G , Beyreuther , K and Kimberlin , RH . 1986 . The major polypeptide of scrapie‐associated fibrils (SAF) has the same size, charge distribution and N‐terminal protein sequence as predicted for the normal brain protein . EMBO J , 5 : 2591 – 7 .
   PubMed Web of Science ®Google Scholar
• Hsiao , KK , Scott , M , Foster , D , Groth , DF , Dearmond , SJ and Prusiner , SB . 1990 . Spontaneous neurodegeneration in transgenic mice with mutant prion protein . Science , 250 : 1587 – 90 .
   PubMed Web of Science ®Google Scholar
• Hunter , N , Foster , JD , Benson , G and Hope , J . 1991 . Restriction fragment polymorphisms of the scrapie‐associated fibril protein (PrP) gene and their association with susceptibility to natural scrapie in British sheep . J Gen Virol , 72 : 1287 – 92 .
   PubMed Web of Science ®Google Scholar
• Hunter , N , Foster , JD , Dickinson , AG and Hope , J . 1989 . Linkage of the gene for the scrapie‐associated fibril protein (PrP) to the Sip gene in Cheviot sheep . Vet Record , 124 : 364 – 6 .
   PubMed Web of Science ®Google Scholar
• Hunter , N , Hope , J , McConnell , I and Dickinson , AG . 1987 . Linkage of the serapie‐associated fibril protein (PrP) gene and Sinc using congenic mice and restriction fragment length polymorphism analysis . J gen Virol , 68 : 2711 – 6 .
   PubMed Web of Science ®Google Scholar
• Kascsak , RJ , Rubinstein , R Merz , PA . 1987 . Mouse polyclonal and monoclonal antibody to scrapie‐associated fibril proteins . J Virol , 61 : 3688 – 93 .
   PubMed Web of Science ®Google Scholar
• Kimberlin , RH . 1982 . Reflections on the nature of the scrapie agent . Trends Biochem Sci , 7 : 392 – 4 .
   Web of Science ®Google Scholar
• Kimberlin , RH . 1986 . Scrapie: how much do we really understand? . Neuropathol Appl Neurobiol , 12 : 131 – 47 .
   PubMed Web of Science ®Google Scholar
• Kimberlin , RH . 1990 . Scrapie and possible relationships with viroids . Seminars in Virol , 1 : 153 – 62 .
   Google Scholar
• Kimberlin , RH and Hope , J . 1987 . Genes and genomes in scrapic . Trends in Genetics , 3 : 117 – 8 .
   Web of Science ®Google Scholar
• Kimberlin , RH , Walker , CA Millson , GC . 1983 . Disinfection studies with two strains of mouse‐passaged scrapie agent . J Neurol. Sci , 59 : 355 – 69 .
   PubMed Web of Science ®Google Scholar
• Latarjet , R . 1979 . “ Inactivation of the agents of scrapie, CJD, and kuru by radiations ” . Edited by: Prusiner , SB and Hadlow , WJ . Vol. 2 , 387 – 407 . Academic Press : Slow transmissible diseases of the nervous system .
   Google Scholar
• McKinley , MP , Bolton , DC and Prusiner , SB . 1983 . A protease‐resistent protein is a structural component of the scrapie prion . Cell , 35 : 57 – 62 .
   PubMed Web of Science ®Google Scholar
• Merz , PA , Rohwer , RG , Kascsak , R , Wisniewski , HM Somerville , RA . 1984 . Infection specific particle from the unconventional slow virus diseases . Science , 225 : 437 – 40 .
   PubMed Web of Science ®Google Scholar
• Merz , PA , Somerville , RA , Wisniewski , HM and Iqbal , K . 1981 . Abnormal fibrils from scrapie‐infected brain . Acta Neuropathol , 54 : 63 – 74 .
   PubMed Web of Science ®Google Scholar
• Meyer , RK , McKinley , MP , Bowman , KA , Braunfeld , MB , Barry , RA and Prusiner , SB . 1986 . Separation and properties of cellular and scrapie prion proteins . Proc Natl Acad Sci USA , 83 : 2310 – 4 .
   PubMed Web of Science ®Google Scholar
• Meyer , N , Rosenbaum , V Schmidt , B . 1991 . Search for a putative scrapie genome in purified prion fractions reveals a paucity of nucleic acids . J Gen Virol , 72 : 37 – 49 .
   PubMed Web of Science ®Google Scholar
• Narang , HK . 1990 . Detection of single‐stranded DNA in scrapie‐infected brains by electron microscopy . J Mol Biol , 216 : 469 – 73 .
   PubMed Web of Science ®Google Scholar
• Narang , HK , Millar , NS , Asher , DM and Gajdusek , DC . 1991 . Increased multimeric mitochondrial DNA in the brain of scrapie‐infected hamsters . Intervirol , 32 : 316 – 24 .
   PubMed Web of Science ®Google Scholar
• Desch , B. , Westaway , D Walchli , M . 1985 . A cellular gene encodes scrapie PrP 27–30 protein . Cell , 40 : 735 – 46 .
   PubMed Web of Science ®Google Scholar
• Parry , HB . 1983 . “ Recorded occurrences of scrapie from 1750 ” . In Scrapic disease in sheep , Edited by: Oppenheimer , DR . 31 – 59 . London : Academic .
   Google Scholar
• Pocchiari , M , Xi , YG , Casaccia , P , Ladogana , A and Masullo , C . 1991 . “ Pharmacological manipulation of scrapie infectivity ” . In Sub‐acute Spongiform Encephalopathies. Procs CEC seminar Edited by: Bradley , R . 187 – 93 .
   Google Scholar
• Prusiner , SB . 1982 . Novel proteinaceous infectious particles cause scrapie . Science , 216 : 136 – 44 .
   PubMed Web of Science ®Google Scholar
• Prusiner , SB . 1989 . Scrapie prions . Annu Rev Microb , 43 : 345 – 74 .
   PubMed Web of Science ®Google Scholar
• Prusiner , SB . 1991 . Molecular biology of prion diseases . Science , 252 : 1515 – 22 .
   PubMed Web of Science ®Google Scholar
• Prusiner , SB . 1991 . Molecular Biology and transgenetics of prion diseases . Crit Rev Biochem Mol Biol , 26 : 397 – 438 .
   PubMed Web of Science ®Google Scholar
• Prusiner , SB . September 1991 . “ Prion Biology ” . In Procs Conf Prion Disc September , London
   Google Scholar
• Prusiner , SB . 1992 . Prion diseases of humans and animals. Procs Int Meeting on Transmiss Spongif Encephalopathies . Heidelberg , in press
   Google Scholar
• Prusiner , SB , Bolton , DC , Groth , DF , Bowman , KA , Cochran , SP and McKinley , MP . 1982 . Further purification and characterization of scrapie prions . Biochemistry , 21 : 6942 – 50 .
   PubMed Web of Science ®Google Scholar
• Prusiner , SB , Füzi , M Scott , M . 1993 . Immunologic and molecular biologic studies of prion proteins in bovine spongi form encephalopathy . J Inf Dis , 167 : 602 – 13 .
   PubMed Web of Science ®Google Scholar
• Prusiner , SB , Gartin , DE , Banner , JR and Cochran , SP . 1979 . “ On the partial purification and apparent hydrophobicity of the scrapie agent ” . In Slow transmissible diseases of the nervous system , Edited by: Prusiner , SB and Hadlow , WJ . 425 – 61 . Academic Press .
   Google Scholar
• Prusiner , SB , Scott , M Foster , D . 1990 . Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication . Cell , 63 : 673 – 86 .
   PubMed Web of Science ®Google Scholar
• Rohwer , RG . 1984 . Scrapie infectious particle is virus‐like in size and susceptibility to inactivation . Nature , 308 : 658 – 62 .
   PubMed Web of Science ®Google Scholar
• Scott , M , Foster , D Mirenda , C. 1989 . Transgenic mice expressing prion protein produce species‐specific scrapie infectivity and amyloid plaques . Cell , 59 : 847 – 57 .
   PubMed Web of Science ®Google Scholar
• Stahl , N and Prusiner , SB . 1991 . Prions and prion proteins . FASEB J , 5 : 2799 – 807 .
   PubMed Web of Science ®Google Scholar
• Taylor , DM . Decontamination of scrapie‐like agents . Sub‐acute Spongiform Encephalopathies, CEC seminar . Edited by: Bradley . pp. 153 – 9 .
   Google Scholar
• Weissmann , C . 1991 . The prion's progress . Nature , 349 : 569 – 71 .
   PubMed Web of Science ®Google Scholar
• Weissmann , C . 1991 . A ‘unified theory’ of prion propagation . Nature , 352 : 679 – 83 .
   PubMed Web of Science ®Google Scholar
• Westaway , D , Goodman , PA , Mirenda , CA , McKinley , MP , Carlson , GA and Prusiner , SB . 1987 . Distinct prion proteins in short and long scrapie incubation period mice . Cell , 51 : 651 – 62 .
   PubMed Web of Science ®Google Scholar
• Xi , YG , Ingrosso , L , Ladogana , A , Masullo , C and Pocchiari , M . 1992 . Amphotericin B treatment dissociates in vivo replication of the scrapie agent from PrP accumulation . Nature , 356 : 598 – 601 .
   PubMed Web of Science ®Google Scholar
• Part of this paper was presented in a special BSE‐session or the 43rd Conference of the European Association for Animal Production, Madrid, September 1992, and will be published in a special issue of Livestock Production Science.
   Google Scholar
• DLO‐Central Veterinary Institute, Dept. of Pathophysiology and epidemiology, P.O.Box 65, 8200 AB Lelystad, the Netherlands, Fax no: 03200–73473.
   Google Scholar