Prion
Similar to Viruses Atypical
Agents
? Small
? Filterable
? Need host cells
? No machinery for energy generation of
protein synthesis
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
? Resistant to or only
partially inactivated
by
1,Formaldehyde
2,Ethanol
3,Glutaraldehyde
4,Ultraviolet and
ionizing irradiation
5,Non-ionic detergents
? Inactivated by
1,Autoclaving
2,5%sodium
hypochlorite
3,Sodium hydroxide
4,Proteases,urea,
other protein
denaturants
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
Simplified model for prion disease,
How can this model explain the sporadic,acquired or
inherited form of the disease?
Why are there differences in prion
diseases?