behavior/neurological
N |
• mice exhibit normal walking, climbing and rearing when unstressed in their home cages
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• motor symptoms are first detected between P17 and P28 with symptoms becoming more persistent between 2 and 6 months
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• caudal hypertension when suspended by tail income mice
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• transient hyperflexion in some mice
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• upon awakening or in novel environments with hyperextension of one or both hindlimbs
• hypertension response when swimming
• dystonic symptoms during forced treadmill running
• however, mice exhibit normal walking, climbing and rearing when unstressed in their home cages
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• hindlimb slips on a beam with very affected mice falling off the beam
• increased falling on a rotarod
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• hypertension response when swimming
• slower swimming speed than wild-type mice
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• wide-spreading hindlimbs during sitting
• dystonic symptoms during forced treadmill running
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• dystonic symptoms during forced treadmill running
• however, gait is normal on running wheel in home cage
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nervous system
N |
• mice exhibit normal brain, spinal cord and spinal root morphology
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• during abnormal postures, deep cerebellar nuclei and Purkinje cells exhibit irregularly firing neurons with reduced average firing rates and high-frequency bursts compare3d with wild-type cells
• however, firing rates are normal during normal postures
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limbs/digits/tail
muscle
• upon awakening or in novel environments with hyperextension of one or both hindlimbs
• hypertension response when swimming
• dystonic symptoms during forced treadmill running
• however, mice exhibit normal walking, climbing and rearing when unstressed in their home cages
|