mortality/aging
• all mice die by 4 weeks
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behavior/neurological
• at P14, mice exhibit limb hyperkinesias such as ballism and chorea, tonic contractions of the neck and trunk leading to upward deviation of the head and lumbar spine region, and hindlimb clasping unlike wild-type mice
• involuntary movements emerge when mice attempt voluntary movement and prevent the latter
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• at P14, mice exhibit hindlimb clasping unlike wild-type mice
• however, treatment with MK801 ameliorates clasping and convulsions
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• at P14, mice exhibit dystonic posture with forelimbs and hindlimbs extended unlike in wild-type mice
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• mice are unable to walk throughout their lives
• involuntary movements emerge when mice attempt voluntary movement and prevent the latter
• by 4 weeks, mice exhibit rigidity and akinesia unlike wild-type mice
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nervous system
• in the striatum
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• mice exhibit an increase in NMDAR excitatory postsynaptic current (EPSC) to AMPAR EPSC ratio compared with wild-type mice
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growth/size/body
• at P14
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• by P14
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muscle