Analysis Tools
mortality/aging
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• 62% die by weaning and, with rare exception, the remainder die by 35-40 days of age probably from a lethal convulsive episode
• Background Sensitivity: only an occasional mutant on the SJL background survives beyond 6 months
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• more than half of the homozygotes die before weaning
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behavior/neurological
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• mutant mice are easily recognizeable from normal siblings by 11-14 days of age based on their ataxic gait and smaller size
• ataxia is moderate to severe most prominent in the hindlimbs
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abnormal gait
(
J:43429
)
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• affected mice have a wide-based gait
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• Background Sensitivity: spike-wave discharges from homozygotes are detected at 4-5 weeks but are brief (less than 1.5 sec) and rare (1-4/hour) and do not progress in duration or frequency when assessed at 2-3 months of age; these are not detected in mice surviving beyond months
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• mutants undergo rare spontaneous generalized tonic-clonic seizure episodes as early as 14 days og age; seizures are usually of less than one minute and are preceded by several seconds of wild running
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nervous system
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• Background Sensitivity: spike-wave discharges from homozygotes are detected at 4-5 weeks but are brief (less than 1.5 sec) and rare (1-4/hour) and do not progress in duration or frequency when assessed at 2-3 months of age; these are not detected in mice surviving beyond months
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• mutants undergo rare spontaneous generalized tonic-clonic seizure episodes as early as 14 days og age; seizures are usually of less than one minute and are preceded by several seconds of wild running
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growth/size/body
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• affected mice are recognizeable from normal siblings at 11-14 days of age based on slightly smaller size and ataxic gait
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