mortality/aging
• mice die at birth due to respiratory failure
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nervous system
• axons emanating from the midbrain and cranial nerves exhibit descending projections that fail to project through the hindbrain altogether and the central projections of the vagal complex projects aberrantly within the hindbrain compared with wild-type mice
• mice exhibit defects (highly defasciculated, patchy and discontinuous) in the formation of the dorsal funiculus by the central projections of the dorsal root ganglia sensory neurons compared with wild-type mice
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• axons emanating from the midbrain and cranial nerves exhibit descending projections that fail to project through the hindbrain altogether and the central projections of the vagal complex projects aberrantly within the hindbrain compared with wild-type mice
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• endfoot detachment
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• axons emanating from the midbrain and cranial nerves exhibit descending projections that fail to project through the hindbrain altogether and the central projections of the vagal complex projects aberrantly within the hindbrain compared with wild-type mice
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• at E11.5, commissural axons exhibit robust postcrossing trajectory defects with failure to project to the lateral portion of the funiculus and altered lateral and ventral funiculi ratio compared with wild-type mice
• at E13, a large number of commissural axons project abnormally within the floor plate unlike in wild-type mice
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• neuronal heterotopias
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• mice exhibit defects (highly defasciculated, patchy and discontinuous) in the formation of the dorsal funiculus by the central projections of the dorsal root ganglia sensory neurons compared with wild-type mice
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respiratory system
• at birth
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cellular
• axons emanating from the midbrain and cranial nerves exhibit descending projections that fail to project through the hindbrain altogether and the central projections of the vagal complex projects aberrantly within the hindbrain compared with wild-type mice
• mice exhibit defects (highly defasciculated, patchy and discontinuous) in the formation of the dorsal funiculus by the central projections of the dorsal root ganglia sensory neurons compared with wild-type mice
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• axons emanating from the midbrain and cranial nerves exhibit descending projections that fail to project through the hindbrain altogether and the central projections of the vagal complex projects aberrantly within the hindbrain compared with wild-type mice
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• endfoot detachment
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• at E11.5, mice exhibit progressive fragmentation of the basement membrane surrounding the spinal cord which is accompanied by detachment of radial neuroepithelial endfeet from the basal surface unlike wild-type mice
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