Analysis Tools
behavior/neurological
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• circling mice show no acoustic startle responses to startle stimuli of 80 or 120 dB
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• all circling mice fail to orient themselves while swimming, spiral underwater, and are unable to keep their nose and tail above water
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head shaking
(
J:73719
)
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• head shaking is first noticable at 7 days of age and increases over the next 3-4 days
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• circling mice display a reduced rearing score relative to wild-type mice (12.67 vs 56.17, respectively)
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hyperactivity
(
J:73719
)
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• all mice are born normal but gradually become hyperactivite beginning at ~7 days of age
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• mice run in tight circles, esp. when disturbed or placed in strange surroundings, with no preference for any particular direction
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• female circling mice display cannibalism towards heterozygous offspring
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hearing/vestibular/ear
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• at 15 weeks, circling mice display morphological abnomalities of the inner ear
• in contrast, the saccule, utricle, semicircular canals, vestibular hair cells and Scarpa ganglion cells remain intact
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• at 15 weeks, Rosenthal canal appears empty due to severe degeneration of the axons between the hair cells and the spiral ganglion cells
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• at 15 weeks, OHCs are completely lost
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• organ of Corti degeneration is noted at 100 days of age
• however, supporting cells and the stria vascularis remain intact
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• circling mice are totally deaf, as shown by absence of acoustic startle responses at all frequencies tested
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growth/size/body
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• at P7-P100, mutant mice weigh 20%-25% less than wild-type littermates
• however, both viability and fertility remain normal
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nervous system
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• at 15 weeks, OHCs are completely lost
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• at 15 weeks, spiral ganglion cells are almost completely degenerated
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