Analysis Tools
nervous system
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• increase in the number of cells arrested at the promyelinating stage
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• about a 1.5-fold increase in nuclei density is seen in nerves with myelin abnormalities
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• about a 19% reduction in axon diameter and a 23% reduction in nerve fiber diameter resulting an increased G ratio suggesting dysmyelination
• pockets of loose basal lamina are seen in cross sections of axons and Schwann cells are seen through 3 weeks of age
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• at 3 weeks of age, tomacula, sausage-like myelin thickenings, are seen on the sciatic nerve
• paranodal tomacula are most common but internodal tomacula are also seen
• fewer tomacula are seen in adults
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• fewer tomacula and an increase in demyelinating profiles are seen in adults
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behavior/neurological
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• seen by 2 weeks of age
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• hindered gait by 2 weeks of age
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• distal muscle weakness progresses to hindlimb paralysis with age
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• impaired ability to mate
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muscle
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• distal
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growth/size/body
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• by 3 weeks of age weigh about 2g less than wild-type littermates
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