mortality/aging
• mice die at E13.5 similar to Phox2btm1Jbr homozygotes
• treatment of mothers with noradrenergic agonists rescues pup survival up to birth
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nervous system
• authors state that mice exhibit a similar to identical phenotype as that observed in Phox2btm1Jbr homozygotes
• motorneuron differentiation is partially rescued up to the level of the caudal hindbrain
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• the enteric nervous system is absent as in Phox2btm1Jbr homozygotes
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• similar to Phox2btm1Jbr homozygotes, mice exhibit a noradrenergic deficit
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• at later stages of embryonic development, the facial motor nucleus is smaller than in wild-type mice and located in a more anterior position
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• despite an early partial rescue of the noadrenergic deficit, at E 13.5 no locus ceruleus (LC) neurons are present
• however, differentiation of a subpopulation of LC neurons is rescued compared to in Phox2btm1Jbr homozygotes
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• disruptions in brachio-viscero-motor neuron differentiation observed in Phox2btm1Jbr homozygotes are partially rescued but proper migration of facial brachimotor neurons is still defective
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• unlike in Phox2btm1Jbr homozygotes, peripherin+ and Ret+ cells are still present at E16.5 in the ninth and tenth cranial nerve complex although they are less densely packed than in wild-type mice
• atrophy of the ninth and tenth cranial nerve complex is about 72% of the surface area measured
• mice lack dorsal motor nuclei of the vagus and noradrenergic neurons
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• at E11.5
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• the dorsal motor nucleus of the vagus is absent
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homeostasis/metabolism
• similar to Phox2btm1Jbr homozygotes, mice exhibit a noradrenergic deficit
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cardiovascular system
• authors state that mice exhibit a similar to identical phenotype as that observed in Phox2btm1Jbr homozygotes
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