Download Transmissible Spongiform Encephalopathies (Prion disorders)

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Transcript
Transmissible
Spongiform
Encephalopathies
(Prion disorders)
Forms of Transmissible
Spongiform Encephalopathy
• Scrapie
• Creutzfeldt-Jakob disease
• Gerstmann-Straussler-Scheinker
syndrome
• Kuru
• BSE
All associated with
PRIONS
• vCJD
• nvCJD
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