Analysis Tools
mortality/aging
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• only 7.8% of live births from heterozygote matings are homozygous
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• all homozygotes die before weaning, usually between 8 and 16 days of age
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growth/size/body
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• significantly under sized at 1 week of age
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• fail to grow after birth at the normal rate
• normal size at birth
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behavior/neurological
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• attempts by 12 day old mice to move were uncoordinated
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skeleton
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• abnormal joint contracture of both the fore- and hind-limb
• sometimes observed as early as birth and readily observable by P4
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nervous system
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• flattened perineural fibroblasts appear in axons as early as 4 days of age
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• development delayed at 1 day of age
• arrested at promyelinating stage
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• very few axons sorted away from axon bundles
• eventually sorted axons and form basal lamina between 4 and 12 days of age
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• no Remak bundles observed at 4-12 days of age
• axon numbers are much reduced at 8 days
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• no myelination in peripheral regions whereas myelin is present centrally
• central projections of myelin sheath outward from central areas into more peripheral regions of the nerve
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• reduced in size
• hypomyelinated
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• sciatic nerve hypomyelinated
• optic nerve myelination normal
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cellular
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• flattened perineural fibroblasts appear in axons as early as 4 days of age
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