Influence of Breed and Genotype on the Onset and Distribution of Infectivity and Disease-associated Prion Protein in Sheep Following Oral Infection with the Bovine Spongiform Encephalopathy Agent

2015 ◽  
Vol 152 (1) ◽  
pp. 28-40 ◽  
Author(s):  
G. McGovern ◽  
S. Martin ◽  
M. Jeffrey ◽  
S.J. Bellworthy ◽  
J. Spiropoulos ◽  
...  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Prion Protein ◽  
Oral Infection ◽  
Spongiform Encephalopathy ◽  
Bovine Spongiform Encephalopathy Agent

scholarly journals Complex proteinopathy with accumulations of prion protein, hyperphosphorylated tau, α-synuclein and ubiquitin in experimental bovine spongiform encephalopathy of monkeys

2014 ◽  
Vol 95 (7) ◽  
pp. 1612-1618 ◽  
Author(s):  
Pedro Piccardo ◽  
Juraj Cervenak ◽  
Ming Bu ◽  
Lindsay Miller ◽  
David M. Asher
Keyword(s):  
Neurodegenerative Diseases ◽  
Bovine Spongiform Encephalopathy ◽  
Prion Protein ◽  
Common Mechanism ◽  
Spongiform Encephalopathy ◽  
Hyperphosphorylated Tau ◽  
Jakob Disease ◽  
Spongiform Degeneration ◽  
Bovine Spongiform Encephalopathy Agent ◽  
Classical Bovine Spongiform Encephalopathy

Proteins aggregate in several slowly progressive neurodegenerative diseases called ‘proteinopathies’. Studies with cell cultures and transgenic mice overexpressing mutated proteins suggested that aggregates of one protein induced misfolding and aggregation of other proteins as well – a possible common mechanism for some neurodegenerative diseases. However, most proteinopathies are ‘sporadic’, without gene mutation or overexpression. Thus, proteinopathies in WT animals genetically close to humans might be informative. Squirrel monkeys infected with the classical bovine spongiform encephalopathy agent developed an encephalopathy resembling variant Creutzfeldt–Jakob disease with accumulations not only of abnormal prion protein (PrPTSE), but also three other proteins: hyperphosphorylated tau (p-tau), α-synuclein and ubiquitin; β-amyloid protein (Aβ) did not accumulate. Severity of brain lesions correlated with spongiform degeneration. No amyloid was detected. These results suggested that PrPTSE enhanced formation of p-tau and aggregation of α-synuclein and ubiquitin, but not Aβ, providing a new experimental model for neurodegenerative diseases associated with complex proteinopathies.

scholarly journals Enhanced Virulence of Sheep-Passaged Bovine Spongiform Encephalopathy Agent Is Revealed by Decreased Polymorphism Barriers in Prion Protein Conversion Studies

2013 ◽  
Vol 88 (5) ◽  
pp. 2903-2912 ◽  
Author(s):  
J. Priem ◽  
J. P. M. Langeveld ◽  
L. J. M. van Keulen ◽  
F. G. van Zijderveld ◽  
O. Andreoletti ◽  
...  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Prion Protein ◽  
Spongiform Encephalopathy ◽  
Bovine Spongiform Encephalopathy Agent

Risk of oral infection with bovine spongiform encephalopathy agent in primates

The Lancet ◽  
2005 ◽  
Vol 365 (9461) ◽  
pp. 781-783 ◽  
Author(s):  
Corinne Ida Lasmézas ◽  
Emmanuel Comoy ◽  
Stephen Hawkins ◽  
Christian Herzog ◽  
Franck Mouthon ◽  
...  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Oral Infection ◽  
Spongiform Encephalopathy ◽  
Bovine Spongiform Encephalopathy Agent

Risk of oral infection with bovine spongiform encephalopathy agent in primates

The Lancet ◽  
2005 ◽  
Vol 365 (9461) ◽  
pp. 781-783 ◽  
Author(s):  
C LASMEZAS ◽  
E COMOY ◽  
S HAWKINS ◽  
C HERZOG ◽  
F MOUTHON ◽  
...  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Oral Infection ◽  
Spongiform Encephalopathy ◽  
Bovine Spongiform Encephalopathy Agent

scholarly journals No abnormal prion protein detected in the milk of cattle infected with the bovine spongiform encephalopathy agent

2006 ◽  
Vol 87 (8) ◽  
pp. 2433-2441 ◽  
Author(s):  
Sally J. Everest ◽  
Leigh T. Thorne ◽  
Jeremy A. Hawthorn ◽  
Russell Jenkins ◽  
Clare Hammersley ◽  
...  
Keyword(s):  
Oral Dose ◽  
Single Oral Dose ◽  
Bovine Spongiform Encephalopathy ◽  
Prion Protein ◽  
Spongiform Encephalopathy ◽  
Lactation Period ◽  
The Third ◽  
Lactating Cows ◽  
Confirmatory Assay ◽  
Bovine Spongiform Encephalopathy Agent

Milk specimens were collected from lactating cows that had previously been challenged with bovine spongiform encephalopathy (BSE)-infected brain at 4–6 months of age. One group of 10 animals received a single oral dose of 100 g, a second group received 1 g and the third was made up of unexposed controls. The cows were inseminated artificially, and calved at approximately 2 years of age and annually thereafter. Milking was done within the first week following calving and at 10-weekly intervals during the lactation period. Specimens were centrifuged to obtain a fraction enriched for somatic cells and these fractions were analysed for disease-associated, abnormal prion protein (PrPBSE) by using a modified commercial BSE ELISA and a different confirmatory assay. No abnormal prion protein has so far been identified in the cell fraction of milk from cattle incubating BSE by using these methods at their limits of detection.

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