nervous system
N |
• mice exhibit normal generation of the neuronal population that relay motor output signals to the periphery
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• in peripherin+, Isl1+ and Isl1+/peripherin+ ventral motor neurons
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• abnormal projection of proprioceptive sensory axons
• at E17.5, projections of proprioceptive afferents into the ventral termination zone (group Ia afferents) is nearly absent compared with control mice
• however, the population of proprioceptive sensory neuron cell bodies in the dorsal root ganglion is normal
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behavior/neurological
• severe defect by P15 affecting hindlimb locomotion
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• hindlimb gait described as a prolonged step cycle period with overall increased amplitude of the locomotive rhythm as early as P15 and persistent until death
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