Analysis Tools
mortality/aging
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• few mice survive past 3 weeks of age
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nervous system
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• spindle density in the soleus, the medial gastrocnemius, plantaris and lumbrical muscles is reduced 50% compared to in wild-type mice
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• 31% of soleus muscles are devoid of muscle spindles
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• mice exhibit a 78% loss in sensory neurons in the dorsal root ganglion
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• mice exhibit a loss of 82% myelinated fibers in the dorsal root ganglion
• mice exhibit a loss of 75% of dorsal root ganglion neurons
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• at E14.5, Ia neuron projections in the dorsal root ganglion are severely reduced
• mice exhibit a 90% loss of proprioreceptive neurons in the dorsal root ganglion
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• at E14.5, L4 is smaller than in wild-type mice due to a 12.1-fold increase in apoptosis
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behavior/neurological
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• mice exhibit dystonia during the first two weeks after birth but not during adulthood
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• mice exhibit ataxia during the first two weeks after birth but not during adulthood
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abnormal gait
(
J:53825
)
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• mice exhibit an unstable gait and mild posturing of extremities during the first two weeks after birth but not during adulthood
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muscle
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• mice exhibit dystonia during the first two weeks after birth but not during adulthood
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• muscles exhibit fewer myelinated fibers than in wild-type mice (26+/-2 compared to 76+/-2 in wild-type mice)
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• spindle density in the soleus, the medial gastrocnemius, plantaris and lumbrical muscles is reduced 50% compared to in wild-type mice
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• 31% of soleus muscles are devoid of muscle spindles
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muscle spasm
(
J:53825
)
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• mice that survive longer than 3 weeks exhibit flexor spasms and uplifting of hindlimbs during weeks 3 to 4
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growth/size/body
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• mice weight 10% to 30% less than wild-type mice
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