mortality/aging
• mice die soon after birth due to respiratory failure
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nervous system
• at E10.5, the cell fate specification of dorsal progenitor 1 through 3 domains of neural progenitor cells is altered such that Pou4f1 (Brn3a)+ dorsal interneuron 2 and dorsal interneuron 3 neurons are respecified to Pax2+ dorsal interneuron 4 neurons
• at E18.5, Phox2b+ neurons derived from the caudal rhombic lip of dorsal progenitor 3 domain is inhibited and the formation of brainstem (nor) adrenergic centers and first-order relay visceral sensory neuron is disrupted as determined by marker expression
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• expression of Zic1 in the extramural migratory stream is decreased compared to in wild-type mice
• expression of Pou4f1 (Brn3a) in the intramural migratory stream is lost
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• at E14.5 through E18.5, Pou4f1 (Brn3a)+ climbing fibers are not detected in the inferior olive nucleus unlike in wild-type mice indicating a loss of these neurons
• at E18.5, Nissl staining fails to detect the inferior olivary complex structure
• however, no ectopic climbing fibers or inferior olivary complexes are detected
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• at E14.5 and E16.5, formation of the mossy fiber nuclei is inhibited unlike in wild-type mice as determined by expression of Barhl1 and Zic1
• unlike in wild-type mice, lateral reticular nuclei mossy fiber neurons are not detected
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respiratory system
homeostasis/metabolism
cellular
• at E10.5, the cell fate specification of dorsal progenitor 1 through 3 domains of neural progenitor cells is altered such that Pou4f1 (Brn3a)+ dorsal interneuron 2 and dorsal interneuron 3 neurons are respecified to Pax2+ dorsal interneuron 4 neurons
• at E18.5, Phox2b+ neurons derived from the caudal rhombic lip of dorsal progenitor 3 domain is inhibited and the formation of brainstem (nor) adrenergic centers and first-order relay visceral sensory neuron is disrupted as determined by marker expression
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