Analysis Tools
behavior/neurological
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• severe balancing problems begin around 3 weeks of age
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bradykinesia
(
J:76481
)
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• episodes of sudden arrest begin around 3 weeks of age
• increasingly frequent episodes of random, involuntary movements by 26 days of age
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• repetitive movements begin around 3 weeks of age
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growth/size/body
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• mice did not gain weight after day 20, and by day 35, their weight was less than half that of unaffected littermates
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immune system
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• mice have elevated expression of Il1b, Il6, Il10, Il12 in the CNS, with highest expression in regions that exhibit neurodegeneration
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• mice have elevated expression of Tnf in the CNS, with highest expression in regions that exhibit neurodegeneration
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muscle
muscle spasm
(
J:76481
)
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• increased frequency of episodes of sudden involuntary limb movements by 26 days of age
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nervous system
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• astrogliosis and microglia activation in the striatum at 23 days of age
• in the N. amygdaloides corticalis, astrogliosis beginning at 26 days and microglia activation beginning at 31 days
• gliosis of cervical spinal cord motoneurons after day 30
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• coronal sections from mice between the ages of 20 to 40 days showed progressive neurodegeneration, with the most extensive degeneration in the striatal neurons beginning around 23 days
• ~50% reduction in midstriatal neurons at 39 days of age
• clumps of chromatin exhibited a "bulls eye profile" in striatal neurodegeneration at 39 days
• laddering degradation of striatal DNA at 23 days and 39 days
• nuclear morphology of affected neurons showed characteristics of apoptosis
• cytoplasmic vacuoles and early disintegration of mitochondria in degenerating striatal neurons
• neuronal cell death was not detected in the cerebellum
• transgenic overexpression of human BCL2 in neurons did not prevent striatal cell loss
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• sporadic degeneration and gliosis of cervical spinal cord motoneurons were observed after day 30
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homeostasis/metabolism
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• mice have elevated expression of Il1b, Il6, Il10, Il12 in the CNS, with highest expression in regions that exhibit neurodegeneration
|
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• mice have elevated expression of Tnf in the CNS, with highest expression in regions that exhibit neurodegeneration
|
