nervous system
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• in the lumbar spinal cord, motoneuron loss is increased by 23% and 26% at P1 and P9, respectively
• in the thoracic spinal cord, motoneuron loss is increased by 29% and 43% at P1 and P9, respectively
• in the brachial spinal cord, motoneuron loss is increased by 30% and 40% at P1 and P9, respectively
• in the brainstem nuclei, facial motorneuron loss is increased by 24% and 22% at P1 and P9, respectively, by 20% at 4 weeks, and by 16% and 17% at 3 and 6 months, respectively, while hypoglossal motorneuron loss is increased by 23% at both P1 and P9
• however, no significant differences in motoneuron numbers are detected in the lumbar spinal cord at E13.5-E14.5 or in the facial nucleus at E15 relative to wild-type mice
• in addition, axotomized homozygotes show no significant further loss of motorneurons in the facial nucleus at 2 or 6 months relative to wild-type mice
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behavior/neurological
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• at 4 months of age, homozygotes display a significant reduction in grip strength relative to wild-type littermates
• however, loss of muscle strength does not result in any other obvious behavioral deficits
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