Analysis Tools
behavior/neurological
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• display spastic leg extension and enhanced clasping reflex when suspended by the tail
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• tend to stagger
• fall more rapidly from a rotarod
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• hind limb rigidity during their first 3 weeks of life
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nervous system
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• display a range of abnormalities including tomacula, comma shaped myelin outfolds and redundant myelin profiles
• in some cases the tomacula are accompanied with myelin degeneration around the axon and axonal displacement
• redundant outfoldings are present in the Schwann cell cytoplasm, between the myelin sheath and axon, and are located near paranodes and Schmidt-Lanterman incisures
• at 2 weeks of age detachment of the myelin sheath from the axon at the inner mesaxon and the presence of multiple inner myelin lips are seen
• abnormalities are detected during the first postnatal week
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• abnormal splitting of the paranodal loops
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• at P1 and P3 fewer axons display myelin profiles compared to controls
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