behavior/neurological
• forelimb clasping
• action-induced forepaw clenching during tail suspension
|
• mice develop spontaneous abnormal movements by second postnatal week
• unilateral twisted hind paw
• bilateral twisted hind paws
• prolonged stiff extension of hind limbs
• abnormal toe postures
• tail extension
|
• mice exhibit an increase in the number of footslip/cross in beam crossing
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• 4 of 7 mice show twisted hindpaws at P15
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growth/size/body
• mice are significantly smaller than littermate controls
|
• reduced postnatal growth
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muscle
• mice develop spontaneous abnormal movements by second postnatal week
• unilateral twisted hind paw
• bilateral twisted hind paws
• prolonged stiff extension of hind limbs
• abnormal toe postures
• tail extension
|
nervous system
• loss of red nucleus by P56
|
• minimal gliosis is observed in spinal cord
(J:213785)
• reactive gliosis is observed at P56 in deep layers of the sensorimotor cortex, ventral posterior thalamus, globus pallidus, deep cerebellar nuclei, red nucleus and facial nerve nuclei and is less severe than gliosis found in null mice
(J:213785)
• reactive gliosis in the cortex and thalamus
(J:288753)
|
• abnormal accumulation of perinuclear ubiquitin
|
• striatal cholinergic interneuron degeneration
|
vision/eye
• squinty eyes
|