Prion

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Prion
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Similar to Viruses Atypical Agents

• Small
• Filterable
• Need host cells
• No machinery for energy generation of protein
  synthesis

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Different from viruses

• No dectectable virions in infected tissues
• No detectable virions in purified infectious
  material
• If nucleic acid is present, very small
• Very resistant

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• Resistant to or only partially inactivated by
• Formaldehyde
• Ethanol
• Glutaraldehyde
• Ultraviolet and ionizing irradiation
• Non-ionic detergents

• Inactivated by
• Autoclaving
• 5sodium hypochlorite
• Sodium hydroxide
• Proteases, urea, other protein denaturants

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Pathogensis

• Kuru
• CJD(Creutzfeldt Jakob disease)
• GSS(Gerstmann Straussler-Scheinker syndrome)
• FFI(Fetal familial insomnia)
• Scrapie(sheep and goats)
• Bovine spongiform encephalopathy
• Transmissible mink encephalopathy
• Chronic wasting disease of mule deer

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Simplified model for prion disease.
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How can this model explain the sporadic, acquired
or inherited form of the disease?
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Why are there differences in prion diseases?
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