Analysis Tools
nervous system
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• mutant Schwann cells display arrested radial growth and reduced longitudinal growth
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• there is a 1.9-fold increase in the number of Schwann cells in mutant nerves compared with controls
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• dramatic reduction in the cross-sectional area of peripheral mutant nerves which is first significant at P7 although these are not significantly different from controls until P21
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• in unmyelinated, large diameter axons, in addition to some vacuolation and redundant basal lamina, the axonal membrane was highly irregular in outline
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• internodes in mutant quadriceps nerves at P21 show a wider range of lengths compared with control nerves and mean values are reduced
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• electron microscopy reveals that myelin is consistently thinner than normal; this aberrant ensheathment persists from P7 until P90; growth of the myelin sheath is retarded and/or arrested
• other derangements, such as myelin outfoldings, were also observed at low frequency in the mutant from P7 onwards but not in controls
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• in P21 sciatic nerve lysates, there is a decrease in the amount of the major peripheral myelin protein P0, suggesting a deficit in myelin production
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• nerve conduction velocities of mutant nerves are severely reduced from 38.7 +/- 0.7 m/s to 11.7 +/- 1.5 m/s
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behavior/neurological
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• poorer performance in the Rotarod test at P42; test was performed at two different speeds, 24 and 32 rpm
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