behavior/neurological
• mice display a continued high activity and failure to habituate to a novel environment
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• during the hidden portion of the water maze, mutants require a longer distance and time to find the platform compared to controls
• however, during the cued portion of the water maze, mutants perform similarly to controls
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• on day 7, during the probe portion of the water maze, mice show impairments in memory retention
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• 6-8 and 12-14 month old mice, but not 3-4 month old mice, show impairments when crossing a round beam, with a higher rate of errors
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• increase in total activity beginning at 3-4 months of age with a time dependent increase 6-8 and 12-14 months of age
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nervous system
• 8-10 month old mice show alterations in the mitochondria of the hippocampus dentate gyrus, with some enlarged and elongated mitochondria that display disorganized cresta
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• decrease in the neocortical volume
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• age dependent increase in tau protein aggregation in layer II-III and V pyramidal neurons in the neocortex and pyramidal and granular cells in the hippocampus
• tau accumulates in synaptohysin positive presynaptic terminals and in MAP2 immunoreactive dendrites
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• dendrites show accumulation of lamellar bodies and straight filamentous structures
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• reduction in the number of neurons in the CA1 and dentate gyrus of the hippocampus
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• intra-neuronal inclusions in the frontal cortex
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• increase in GFAP immunoreactivity in the neocortex and hippocampus and glial cells are detected in the hippocampus indicating neurodegeneration
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• axonal dystrophy in 8-10 month old mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Pick's disease | DOID:11870 |
OMIM:172700 |
J:229181 |