nervous system
• mice show severe demyelinating neuropathy
• nerves show a strong reduction of myelinated axons at P24, with an increase in the number of sorted myelin-competent axons that are not enwrapped by myelin
• cells with engulfed myelin inclusions, indicative of ongoing demyelination, are commonly seen in nerves at P24
• however, the number of sorted axons and numbers of structural myelin aberrations are not changed
• no morphological changes are seen in tibial nerves or sciatic nerves at P5, indicating normal radial axonal sorting and correct onset of myelination
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• electrophysiology shows decreased compound muscle action potential amplitude at P24
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• electrophysiology shows reduced motor nerve conduction velocity at P24
• compound sensory nerve conduction velocity and compound sensory amplitude are not detectible in P24 mice
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