Analysis Tools
mortality/aging
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• 51 of 52 mutants die before 1 year of age, with a median survival of 75 days
• administration of a ketogenic diet prolongs life span of mutants; females on the ketogenic diet have a median survival of 334 days while males have a median survival of 154 days
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behavior/neurological
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• 19 of 21 mutants experience seizures for about 1 minute immediately prior to death
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• 32% of mutants exhibit handling-induced seizures at a median of 18 days before death
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• seizures are characterized by a progression from wild running to tonic-clonic convulsion to tonic hindlimb extension that ends in death
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nervous system
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• 19 of 21 mutants experience seizures for about 1 minute immediately prior to death
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• 32% of mutants exhibit handling-induced seizures at a median of 18 days before death
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• seizures are characterized by a progression from wild running to tonic-clonic convulsion to tonic hindlimb extension that ends in death
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• mutants exhibit several EEG abnormalities including more interictal discharges and lower interhemispheric coherence compared to wild-type mice
• baclofen, a GABABR-specific agonist, shows impaired ability to induce delta waves on EEG tracings in mutants
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