Analysis Tools
nervous system
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• a subset of mice develop spontaneous progressive signs of neurological illness consistent with prion disease beginning around 400 days of age
• about 60% of mice develop spontaneous disease by 20 months of age
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• infectious prion seeds form spontaneously in the brain
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• brains from spontaneously ill mice exhibit prominent gray matter vacuolation in the hippocampus, cortex, and thalamus
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astrocytosis
(
J:361333
)
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• brains of symptomatic mice exhibit increased astocytic gliosis in regions with vacuolation
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• mice develop prion disease-specific neuropathological changes including spongiform degeneration
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mortality/aging
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• all-cause mortality is increased
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behavior/neurological
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• spontaneously sick mice show variable presence of limb abnormalities and ataxia
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growth/size/body
weight loss
(
J:361333
)
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• in spontaneously sick mice, one of the most common signs of illness is prominent weight loss
• progressive weight loss tends to begin approximately 3-4 weeks before terminal disease
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homeostasis/metabolism
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• mice show progressive accumulation of protease-resistant prion protein in the brain with age
• intracellular prion protein deposition within glial cells is selectively present in the striatum of sick mice
• however, no extracellular PrPSc deposition is seen in brain regions exhibiting spongiform degeneration
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immune system
dermatitis
(
J:361333
)
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• some mice exhibit severe dermatitis characterized by neurotic scratching/overgrooming the head region
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integument
dermatitis
(
J:361333
)
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• some mice exhibit severe dermatitis characterized by neurotic scratching/overgrooming the head region
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skeleton
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• in spontaneously sick mice, one of the most common signs of illness is prominent kyphosis
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| prion disease | DOID:649 | J:361333 | ||
