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Transmissible Spongiform
Encephalopathies (Prion disorders)
Forms of Transmissible Spongiform Encephalopathy
• Scrapie• Creutzfeldt-Jakob disease• Gerstmann-Straussler-Scheinker
syndrome• Kuru• BSE• vCJD• nvCJD
All associated withPRIONS
Animals Effected by Transmissible Spongiform
Encephalopathy
• Sheep, goats, deer, elk, mink, domestic cats, zoo species, cattle and humans
BSE
• First seen in UK in 1986
• Linked to cattle food supplemented with rendered offal from slaughtered sheep
• Perhaps stimulated and amplified by the use of air embolus to slaughter cattle (captive bolt gun)
Both are temporally related to the advent of BSE
Prions• Proteinaceous infectious particle
• Discovered by Dr. Stanley Prusiner
• Prusiner awarded 1997 Nobel prize for physiology/medicine
• Protein composition
• Apparently capable of self reproduction
• Contains no DNA or RNA
• Very resistant to high temperatures and disinfection