Analysis Tools
immune system
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• intracerebral inoculation with bovine spongiform encephalopathy (BSE) prions results in prion disease development with a mean incubation period of 210 days
• mice inoculated with brain homogenate from a variant Creutzfeldt-Jakob disease (vCJD) patient results in disease development with a mean incubation period of 199 days
• secondary passage of vCJD prions in mice reduces the mean incubation period to 122 days
• brains of mice inoculated with BSE or vCJD prions exhibit proteinase K-resistant prion protein
• however, none of the mice inoculated with brain homogenate from sporadic CJD patients exhibiting the MM1, MM2, or VV2 disease subtypes develop clinical signs of prion disease or mice inoculated with the RML strain of mouse-passaged scrapie prions
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nervous system
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• mice show no signs of spontaneous neurological dysfunction up to 600 days of age
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• brains of BSE- and vCJD-inoculated mice exhibit PrPSc deposition in the brain
• however, florid PrPSc plaques are not seen in the brains of vCJD-inoculated mice
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• mice show prominent loss of CA2 and CA3 neurons in the hippocampus upon first passage of BSE prions
• mice show extensive cell loss in all areas of the hippocampus, including the dentate gyrus upon vCJD inoculation
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• brains of BSE- and vCJD-inoculated mice exhibit moderate-to-severe spongiform degeneration and vacuolation upon first passage of the prions
• BSE- and vCJD-inoculated mice show abundant vacuolation in the cerebral cortex
• large clusters of vacuoles and numerous coarse PrPSc deposits are seen in the hippocampus upon secondary passage of the vCJD and BSE isolates
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| variant Creutzfeldt-Jakob disease | DOID:5435 | J:236012 | ||
