Analysis Tools
mortality/aging
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• all mutants die by 24 days of age with a median age of lethality of 18 days
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homeostasis/metabolism
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• ceramide concentration in the brain is decreased about 1.7-fold at P0 and P15, but sphingomyelin levels are consistently increased after birth
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behavior/neurological
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• when placed on their ridge mutants take about 6 seconds to roll over compared to less than 0.5 seconds for control littermates
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• mutants develop severe ataxia with a shuffling gait and strong equilibrium impairments
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• mutants are unable to balance on a rotating rod
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• shuffling gait
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nervous system
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• the dendritic tree shows decreased height and arborization in mutant cerebella
• in culture neurite length and the number of branching points are decreased for Purkinje cells from E15.5 mutant embryos
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• surface area of the axon of the femoral nerve is increased
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• myelin surface area of the femoral nerve is increased and in some mutants splitting of the myelin sheath is seen
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growth/size/body
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• body weight is similar to control littermates at birth, but after birth mutants gain less weight than their control littermates
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