Analysis Tools
nervous system
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• mice develop sense and antisense RNA foci throughout the nervous system and production of repeat-associated non-ATG (RAN) translated dipeptides
• however, mice show no differences in neuromuscular junction innervation or femoral axon counts, no protein aggregation, gliosis, or inflammation in the brain and spinal cord, and no synapse or neuron loss
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cellular
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• neurons show dispersion of nucleolin from the nucleolus suggesting a degree of nucleolar stress
• primary cortical cultures show RNA foci in 73% of cultured neurons and astrocytes
• however, primary cortical neuron cultures show normal frequency of calcium transients and sensitivity to glutamate toxicity
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behavior/neurological
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• mice show no abnormalities in body weight, grip strength, rotarod, open field testing analysis, in the Y-maze assessing memory and novelty-seeking behavior, or in the three-chamber test of sociability and social novelty at 3 and 18 months of age
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