mortality/aging
• mice die at birth due to respiratory failure
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nervous system
• several markers of motor neurons are absent (Lhx4, Lhx1 and Er81) indicating that motor neurons have adopted a axial-innervating identity (medial half of median motor column)
• at E15.5, no Isl1+ cells in the intermediolateral position of the preganglionic motor column (PGC) motor neurons are detected in the thorax compared to 12+/-2 intermediolateral PGC cells in control mice
• fewer motor neurons project to their target
• lateral motor column (LMC) motor neurons fail to migrate from the ventral spinal cord to their normal intermediolateral position
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• axial innervations are increased whereas limb innervations are decreased
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• while axons arbourize extensively the ramus visceralis branch is nearly absent
(J:63774)
• explants exhibit an increased in the number of axons attracted to the dermomyotome compared with explants from wild-type mice
(J:122940)
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respiratory system