Analysis Tools
mortality/aging
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• 10% of mice surviving to weaning die by 12 weeks of age
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• 40% die between birth and weaning
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growth/size/body
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• body weigts are 25% less than wild-type controls, but number may be biased due to many of the smallest mice dying prior to the analysis
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behavior/neurological
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• mice have a detectable postural intention tremor that worsens when mice are suspended by the tail
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• male mice display impaired coordination in the accelerating rotarod test compared to controls
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• surviving mice often exhibit hunched posture
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• mice show reduced locomotor activity compared to wild-type when monitored for activity in their home cage for a 4-hour period, but show greater activity than wild-type over the first 20 minutes of a 60 minute period in a novel test environment
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• surviving mice are hypoactive
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• mice have an exaggerated response to human contact, including vigorous jumping and escape behavior
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• only 1 of 90 pups survived more than 2 days due to impaired nurturing behavior
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nervous system
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• miniature inhibitory postsynaptic currents in hippocampal neurons decay more slowly than in wild-type
• in thalamic neurons, in response to isofluorane, the decay time of miniature inhibitory postsynaptic currents is prolonged by 38% compared to 237% in wild-type
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