mortality/aging
• mice exhibit a more pronounced mortality rate
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behavior/neurological
• mice show disturbances of activity-rest consolidation, showing more activity bouts both during the light and dark cycles compared to wild-type mice, with the duration of these activity bouts reduced during the dark cycle
• mice do not have a rapid transition to high activity with the onset of the dark cycle, the latency to peak activity during the dark cycle is lengthened, and activity remains elevated at the end of the dark cycle and beginning of the light phase
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• mice exhibit enhanced sensitivity to nicotine-induced seizures and nicotine-induced dorsiflexion (Straub tail)
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• mice do not develop a normal proclivity to voluntary wheel running, showing less wheel-running when allowed free access to wheels and indicating impaired natural reward-seeking behaviors
• however, mice show normal spatial learning in the Morris water maze
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• mutants habituate to the novel environment more slowly than wild-type mice
• mice introduced to the open field for 30 minutes each day for 5 days do not show a decline in locomotion on day 2 of the trail like wild-type mice
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• in the elevated plus maze, mutants enter the open arms more often and spend more time in the open arms, indicating decreased anxiety
• however, mutants do not show any differences from wild-type mice in the light/dark box test or in the amount of time spent in the center of the open-field test
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• mice remain on the rotarod longer than wild-type mice
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• mutants show increased levels of activity, traveling over a greater distance during the first 90 minutes in the novel environment of the open field
• in an open-field test, mutants maintain their activity levels during the dark phase compared to wild-type mice that show an abrupt reduction in activity about 8 hours into the dark phase
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• mice exhibit an increase in the number of activity bouts and a higher percentage of total activity during the light phase, suggesting sleep disruption
• mice exhibit more activity bouts with shorter average duration during the dark phase, suggesting they sleep more frequently during the dark cycle
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• mice exhibit enhanced sensitivity to nicotine-induced seizures
• however, spontaneous motor seizures rarely occur
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nervous system
• mice exhibit enhanced sensitivity to nicotine-induced seizures
• however, spontaneous motor seizures rarely occur
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
autosomal dominant nocturnal frontal lobe epilepsy 3 | DOID:0060684 |
OMIM:605375 |
J:228269 |