behavior/neurological
• on rotating rod, mice show an early onset, at >3 weeks of age, and progressive decline of motor performance
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nervous system
astrocytosis
(
J:63836
)
• astrocytic gliosis is observed in anterior horn of spinal cord and in some brain regions; microglial activation is also seen
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• some neurons in anterior horn show a Lewy-like pathology
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• in all muscles examined, presynaptic motor neurons show signs of degeneration
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• neurites show heterogeneous changes including enlargements of proximal and distal neuritic segments, fine or thread-like inclusions, and beaded or spindle-shaped neurites
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• 10-40% of synapses are denervated in some mice and thinning of preterminal nerves, or swellings are detected in at least 50% of innervated synapses; denervation is observed in all muscles examined
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• neurons in brain and some in spinal cord show accumulation of alpha-synuclein protein, similar to Lewy pathology in human brains
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• apparent in spinal roots; nerve fibers show breakdown
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muscle
• muscle contains small angular fibers, indicating neurogenic atrophy
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