nervous system
N |
• despite reduction in the size of the cortex and cerebrum, the overall brain and spinal cord structure are normally organized
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• at 2 and 20 months, mice exhibit microglial activation in the spinal cord
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• the cortex is slightly smaller than in wild-type mice
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• slightly
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• mice exhibit an increase in fiber diameter size
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• mice exhibit an increase in fiber diameter size
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• the spinal cord accumulates autophagic vacuoles
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• neurofilaments accumulate in the cerebellum, brainstem, habenula, and spinal cord unlike in wild-type mice suggesting axonal dystrophy
• degenerating axons with swelling and many vacuoles are observed in the spinal cord
• however, myelination of axons is normal
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• detected by neurofilament antibodies
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• anterograde and retrograde transport are impaired
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behavior/neurological
• motor learning in a rotarod assay is partially impaired compared to in wild-type mice
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• when suspended by the tail, mice exhibit spinning and clasping
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• latency to fall on a rotarod assay is decreased
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• in an open field test
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muscle
• some mice exhibit atrophic fibers in both slow- and fast-twitch muscles
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immune system
• at 2 and 20 months, mice exhibit microglial activation in the spinal cord
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hematopoietic system
• at 2 and 20 months, mice exhibit microglial activation in the spinal cord
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