Journal ArticleDOI
Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier
TLDR
The BSE agent has retained its identity when passaged through a range of species and the 'donor' species has little specific influence on disease characteristics in mice, adding to evidence for an agent-specific informational molecule.Abstract:
Transmissions of bovine spongiform encephalopathy (BSE) from seven unrelated cattle sources have given remarkably uniform disease characteristics in mice, differing from over twenty previous and contemporary transmissions of sheep and goat scrapie. Transmissions to mice of spongiform encephalopathy from six species (including sheep and goats) which have been experimentally or naturally infected with BSE have given similar results to direct BSE transmissions from cattle. Therefore the BSE agent has retained its identity when passaged through a range of species and the 'donor' species has little specific influence on disease characteristics in mice, adding to evidence for an agent-specific informational molecule. On transmission of BSE or scrapie to mice the incubation periods are long compared with subsequent mouse-to-mouse passages (the 'species barrier'). Contributing factors include a low efficiency of infection on interspecies transmission, the apparent failure of intracerebrally injected 'foreign' inoculum to establish infection directly in mouse brain and the selection of variant strains of agent which replicate most readily in the new host species.read more
Citations
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Journal ArticleDOI
Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent
Moira E. Bruce,Robert G. Will,James W. Ironside,I. McConnell,D. Drummond,A. Suttie,L. McCardle,A. Chree,James Hope,Christopher R. Birkett,Simon Cousens,H. Fraser,Christopher J. Bostock +12 more
TL;DR: It is shown that the strain of agent from cattle affected by bovine spongiform encephalopathy (BSE) produces a characteristic pattern of disease in mice that is retained after experimental passage through a variety of intermediate species, providing strong evidence that the same agent strain is involved in both BSE and vCJD.
Journal ArticleDOI
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
TL;DR: Strain characteristics revealed here suggest that the prion protein may itself encode disease phenotype, consistent with BSE being the source of this new disease.
Journal ArticleDOI
Prion Diseases of Humans and Animals: Their Causes and Molecular Basis
TL;DR: The appearance of a novel human prion disease, variant CJD, and the clear experimental evidence that it is caused by exposure to BSE has highlighted the need to understand the molecular basis of prion propagation, pathogenesis, andThe barriers limiting intermammalian transmission.
Journal ArticleDOI
A General Model of Prion Strains and Their Pathogenicity
John Collinge,Anthony R. Clarke +1 more
TL;DR: Recent advances suggest that prions themselves are not directly neurotoxic, but rather their propagation involves production of toxic species, which may be uncoupled from infectivity.
Journal ArticleDOI
Prion Diseases and the BSE Crisis
TL;DR: There is now considerable concern that bovine prions may have been passed to humans, resulting in a new form of CJD.
References
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Journal ArticleDOI
Novel proteinaceous infectious particles cause scrapie
TL;DR: A new term "prion" is proposed to denote a small proteinaceous infectious particle which is resistant to inactivation by most procedures that modify nucleic acids.
Journal ArticleDOI
Mice devoid of PrP are resistant to scrapie
Hansruedi Büeler,Adriano Aguzzi,Andreas W. Sailer,R.-A. Greiner,P. Autenried,Michel Aguet,Charles Weissmann +6 more
TL;DR: These experiments show that PrPC, possibly at close to normal levels, is required for the usual susceptibility to scrapie and that lack of homology between incoming prions and the host's PrP genes retards disease.
Journal ArticleDOI
A novel progressive spongiform encephalopathy in cattle.
G. A. H. Wells,A.C. Scott,C.T. Johnson,R.F. Gunning,R. D. Hancock,Martin Jeffrey,M. Dawson,R. Bradley +7 more
Journal ArticleDOI
Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication.
Stanley B. Prusiner,Michael R. Scott,Dallas Foster,Keh-Ming Pan,Darlene Groth,Carol Mirenda,Marilyn Torchia,Shu-Lian Yang,Dan Serban,George A. Carlson,Peter Hoppe,David Westaway,Stephen J. DeArmond +12 more
TL;DR: The results argue that species specificity of scrapie prions resides in the PrP sequence and prion synthesis is initiated by a species-specific interaction between PrPSc in the inoculum and homologous PrPC.
Journal ArticleDOI
Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques
Michael R. Scott,Dallas Foster,Carol Mirenda,Dan Serban,Frank Coufal,Monika Wälchli,Marilyn Torchia,Darlene Groth,George A. Carlson,Stephen J. DeArmond,David Westaway,Stanley B. Prusiner +11 more
TL;DR: It is argued that the PrP gene modulates scrapie susceptibility, incubation times, and neuropathology, and synthesis of infectious scrapie prions programmed by a recombinant DNA molecule is demonstrated.