mortality/aging
• mice survive 17+/-2 days and likely die due to apnea during tonic extension
• observed seizure fatality rate is 50% and does not increased with age
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nervous system
• mice exhibit a shorter latency to seizure following treatment with flurothyl compared to wild-type mice and heterozygotes
• latency to running bouncing seizures is 40% less than in wild-type mice or heterozygotes
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• mice exhibit bursts of wild running and jumping followed by 5 to 10 seconds of full tonic extension similar to running bouncing seizures (RBS)
• observed seizure fatality rate is 50% and does not increased with age
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• neurons exhibit less depolarized sustained membrane potentials and reduced mean resting input resistance compared to wild-type neurons
• however, treatment with dendrotoxin abolishes the differences in neuron electrophysiology compared to wild-type neurons
• tityustoxin has no effect on neurons electrophysiology unlike in wild-type mice
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• neurons exhibit a higher amplitude threshold than wild-type or heterozygous mice
• almost all neurons have low excitability compared to wild-type neurons
• however, all other action potentials and properties not dependent on potassium signaling are normal
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• neurons fire one action potentials when stimulated with 200-pA compared to 3 fired by wild-type mice
• neurons hypoexcitability does not change depending on the age of mice
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• voltage-clamped inwards rectifier potassium current is larger than in wild-type neurons
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growth/size/body
• at P14, mice weigh 1 g less than wild-type and heterozygous mice
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respiratory system
behavior/neurological
• mice exhibit a shorter latency to seizure following treatment with flurothyl compared to wild-type mice and heterozygotes
• latency to running bouncing seizures is 40% less than in wild-type mice or heterozygotes
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• mice exhibit bursts of wild running and jumping followed by 5 to 10 seconds of full tonic extension similar to running bouncing seizures (RBS)
• observed seizure fatality rate is 50% and does not increased with age
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