nervous system
• in 4-15 month old transgenic mice, there is significant accumulation of SDS-insoluble tau protein in hippocampi, compared to wild-type mice
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• abnormal neurons bearing paired helical filament epitopes are observed in transgenic mice; neurons are smaller and convex in appearance relative to wild-type neurons
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• irregularly-shaped neurons in transgenic mice show characteristics of degenerating neurons, including RNA-accumulation in the cytoplasm, a swollen Golgi network, ruffled plasma and nuclear membranes, and straight fibrils in the cytoplasm similar to those associated with neurofibrillary tangles in human disease
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• the irregularly shaped neurons appear to undergo cell death after the accumulation of phosphorylated tau and RNA, but do not show normal signs of apoptotic death
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• Schaffer collateral-evoked neural response in hippocampal slices is attenuated compared to controls; propagation patterns of neural activity is weaker, producing less voltage spread and smaller amplitudes relative to control slices
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Alzheimer's disease | DOID:10652 | J:100964 | ||
frontotemporal dementia | DOID:9255 |
OMIM:600274 |
J:100964 |