mortality/aging
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• 50% mortality by 29-35 weeks of age
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behavior/neurological
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• mice develop progressive cued memory deficits, with the difference in freezing response to a conditioned tone becoming significantly different at 15 weeks of age
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• mice show a diminished acoustic startle response
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• mice develop progressive ataxia that is apparent at 6 weeks and worsens by 20 weeks
• however, mice do not exhibit seizures or stereotypical behaviors
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• mice exhibit reduced latency to fall in both the rotarod and dowel walk assays
• mice exhibit impaired performance of the marble-burying test, suggesting forelimb incoordination
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• mice develop widely splayed hind limbs after 10 weeks and hind limb retraction after 15 weeks of age
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• mice spend more time interacting with novel partner mice in a social behavior assay
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