Analysis Tools
immune system
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• mice are highly susceptible to prions from patients with sporadic Creutzfeldt-Jakob disease (CJD) that contain the codon 129 methionine polymorphism (129MM) but are less susceptible to classical CJD prions from individuals with the codon 129 valine polymorphism (129VV)
• mice are more resistant to variant CJD 129MM prions, with only 1/14 succumbing to clinical disease at a prolonged incubation period, however all mice inoculated with vCJD exhibit pathological and/or biochemical evidence of prion infection and are positive for type 4 PrPSc, indicating 100% infection rate
• vCJD-inoculated mice develop abundant PRNP (Prp) plaques, many of the florid type of a central plaque core surrounded by a ring of spongiform vacuoles
• BSE prion inoculated mice develop clinical disease and sub-clinical infection, showing PrPSc in the brains of clinically sick and in those not showing clinical signs
• 5/6 of clinically affected and 6/8 of sub-clinically affected BSE-inoculated mice show a distinctive human PrPSc type, corresponding to type 2 PrpSc seen in sporadic and iatrogenic CJD, and exhibit widespread vacuolation and extensive gliosis consistent with spongiform encephalopathy
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nervous system
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• 4/6 of clinically affected and 6/8 of sub-clinically affected BSE-inoculated mice show a distinctive human PrPSc type, corresponding to type 2 PrpSc seen in sporadic and iatrogenic CJD, and exhibit widespread vacuolation and extensive gliosis consistent with spongiform encephalopathy
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