Molecular Analysis of Bovine Spongiform Encephalopathy and Scrapie Strain Variation

1998 ◽  
Vol 178 (3) ◽  
pp. 693-699 ◽  
Author(s):  
Thorsten Kuczius ◽  
Ingrid Haist ◽  
Martin H. Groschup
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Molecular Analysis ◽  
Spongiform Encephalopathy ◽  
Strain Variation ◽  
Scrapie Strain

scholarly journals Comparative Molecular Analysis of the Abnormal Prion Protein in Field Scrapie Cases and Experimental Bovine Spongiform Encephalopathy in Sheep by Use of Western Blotting and Immunohistochemical Methods

2004 ◽  
Vol 78 (7) ◽  
pp. 3654-3662 ◽  
Author(s):  
Stéphane Lezmi ◽  
Stuart Martin ◽  
Stéphanie Simon ◽  
Emmanuel Comoy ◽  
Anna Bencsik ◽  
...  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Prion Protein ◽  
Western Blotting ◽  
Molecular Analysis ◽  
Infected Sheep ◽  
Enzyme Linked Immunosorbent Assay ◽  
Spongiform Encephalopathy ◽  
Protease Cleavage ◽  
Two Samples ◽  
Immunohistochemical Methods

ABSTRACT Since the appearance of bovine spongiform encephalopathy (BSE) in cattle and its linkage with the human variant of Creutzfeldt-Jakob disease, the possible spread of this agent to sheep flocks has been of concern as a potential new source of contamination. Molecular analysis of the protease cleavage of the abnormal prion protein (PrP), by Western blotting (PrPres) or by immunohistochemical methods (PrPd), has shown some potential to distinguish BSE and scrapie in sheep. Using a newly developed enzyme-linked immunosorbent assay, we identified 18 infected sheep in which PrPres showed an increased sensitivity to proteinase K digestion. When analyzed by Western blotting, two of them showed a low molecular mass of unglycosylated PrPres as found in BSE-infected sheep, in contrast to other naturally infected sheep. A decrease of the labeling by P4 monoclonal antibody, which recognizes an epitope close to the protease cleavage site, was also found by Western blotting in the former two samples, but this was less marked than in BSE-infected sheep. These two samples, and all of the other natural scrapie cases studied, were clearly distinguishable from those from sheep inoculated with the BSE agent from either French or British cattle by immunohistochemical analysis of PrPd labeling in the brain and lymphoid tissues. Final characterization of the strain involved in these samples will require analysis of the features of the disease following infection of mice, but our data already emphasize the need to use the different available methods to define the molecular properties of abnormal PrP and its possible similarities with the BSE agent.

scholarly journals Molecular Analysis of the Abnormal Prion Protein during Coinfection of Mice by Bovine Spongiform Encephalopathy and a Scrapie Agent

2001 ◽  
Vol 75 (1) ◽  
pp. 107-114 ◽  
Author(s):  
Thierry G. M. Baron ◽  
Anne-Gaelle Biacabe
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Prion Protein ◽  
Proteinase K ◽  
Spongiform Encephalopathy ◽  
Molecular Features ◽  
Electrophoretic Mobilities ◽  
Scrapie Agent ◽  
Scrapie Strain ◽  
Sheep Scrapie

ABSTRACT Molecular features of the proteinase K-resistant prion protein (PrP res) may discriminate among prion strains, and a specific signature could be found during infection by the infectious agent causing bovine spongiform encephalopathy (BSE). To investigate the molecular basis of BSE adaptation and selection, we established a model of coinfection of mice by both BSE and a sheep scrapie strain (C506M3). We now show that the PrP res features in these mice, characterized by glycoform ratios and electrophoretic mobilities, may be undistinguishable from those found in mice infected with scrapie only, including when mice were inoculated by both strains at the same time and by the same intracerebral inoculation route. Western blot analysis using different antibodies against sequences near the putative N-terminal end of PrP res also demonstrated differences in the main proteinase K cleavage sites between mice showing either the BSE or scrapie PrP res profile. These results, which may be linked to higher levels of PrP res associated with infection by scrapie, were similar following a challenge by a higher dose of the BSE agent during coinfection by both strains intracerebrally. Whereas PrP res extraction methods used allowed us to distinguish type 1 and type 2 PrP res, differing, like BSE and scrapie, by their electrophoretic mobilities, in the same brain region of some patients with Creutzfeldt-Jakob disease, analysis of in vitro mixtures of BSE and scrapie brain homogenates did not allow us to distinguish BSE and scrapie PrP res. These results suggest that the BSE agent, the origin of which remains unknown so far but which may have arisen from a sheep scrapie agent, may be hidden by a scrapie strain during attempts to identify it by molecular studies and following transmission of the disease in mice.

scholarly journals Long-Term Subclinical Carrier State Precedes Scrapie Replication and Adaptation in a Resistant Species: Analogies to Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease in Humans

2001 ◽  
Vol 75 (21) ◽  
pp. 10106-10112 ◽  
Author(s):  
Richard Race ◽  
Anne Raines ◽  
Gregory J. Raymond ◽  
Byron Caughey ◽  
Bruce Chesebro
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Carrier State ◽  
Surgical Instruments ◽  
Spongiform Encephalopathy ◽  
Asymptomatic Carriers ◽  
Resistant Species ◽  
Jakob Disease ◽  
Scrapie Strain ◽  
Contaminated Blood

ABSTRACT Cattle infected with bovine spongiform encephalopathy (BSE) appear to be a reservoir for transmission of variant Creutzfeldt-Jakob disease (vCJD) to humans. Although just over 100 people have developed clinical vCJD, millions have probably been exposed to the infectivity by consumption of BSE-infected beef. It is currently not known whether some of these individuals will develop disease themselves or act as asymptomatic carriers of infectivity which might infect others in the future. We have studied agent persistence and adaptation after cross-species infection using a model of mice inoculated with hamster scrapie strain 263K. Although mice inoculated with hamster scrapie do not develop clinical disease after inoculation with 10 million hamster infectious doses, hamster scrapie infectivity persists in brain and spleen for the life span of the mice. In the present study, we were surprised to find a 1-year period postinfection with hamster scrapie where there was no evidence for replication of infectivity in mouse brain. In contrast, this period of inactive persistence was followed by a period of active replication of infectivity as well as adaptation of new strains of agent capable of causing disease in mice. In most mice, neither the early persistent phase nor the later replicative phase could be detected by immunoblot assay for protease-resistant prion protein (PrP). If similar asymptomatic carriers of infection arise after exposure of humans or animals to BSE, this could markedly increase the danger of additional spread of BSE or vCJD infection by contaminated blood, surgical instruments, or meat. If such subclinical carriers were negative for protease-resistant PrP, similar to our mice, then the recently proposed screening of brain, tonsils, or other tissues of animals and humans by present methods such as immunoblotting or immunohistochemistry might be too insensitive to identify these individuals.

Production of prion gene knockout cow to prevent spontaneous bovine spongiform encephalopathy

2014 ◽  
Author(s):  
Noboru Manabe ◽  
Ichiro Onoyama ◽  
Junyou Li ◽  
Yutaka Sendai ◽  
Yoshito Aoyagi
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Gene Knockout ◽  
Spongiform Encephalopathy ◽  
Prion Gene

Abnormalities of heart rate and rhythm in bovine spongiform encephalopathy

1997 ◽  
Vol 141 (14) ◽  
pp. 352-357 ◽  
Author(s):  
A. R. Austin ◽  
L. Pawson ◽  
S. Meek ◽  
S. Webster
Keyword(s):  
Heart Rate ◽  
Bovine Spongiform Encephalopathy ◽  
Spongiform Encephalopathy

scholarly journals Exploration of genetic factors resulting in abnormal disease in cattle experimentally challenged with bovine spongiform encephalopathy

Prion ◽  
2021 ◽  
Vol 15 (1) ◽  
pp. 1-11
Author(s):  
Sandor Dudas ◽  
Renee Anderson ◽  
Antanas Staskevicus ◽  
Gordon Mitchell ◽  
James C. Cross ◽  
...  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Genetic Factors ◽  
Spongiform Encephalopathy
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