Mouse Genome Informatics
involves: C57BL/6 * SJL
phenotype observed in females
phenotype observed in males
N normal phenotype
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
• abnormal neurons bearing paired helical filament epitopes are observed in transgenic mice; neurons are smaller and convex in appearance relative to wild-type neurons
• 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
• 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
• 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

Mouse Models of Human Disease
Alzheimer Disease; AD 104300 J:100964
Frontotemporal Dementia; FTD 600274 J:100964