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