mortality/aging
• most mice die immediately after birth
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nervous system
• in vitro glial differentiation of neural crest stem cells from multiple regions in the peripheral nervous system (gut and sympathetic ganglia) is impaired compared to when wild-type cells are used
• glial cells fail to adopt mature phenotypes unlike in wild-type mice
• enteric sections exhibit fewer glial cells than in wild-type mice
• fewer satellite glia surround sensory neurons in the dorsal root ganglia compared to in wild-type mice
• satellite glia cells surrounding sensory neurons in the dorsal root ganglia exhibit abnormal morphology compared to in wild-type mice
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• mice exhibit reduced glial cells throughout the peripheral nervous system (including enteric glia, dorsal root ganglia, and sympathetic ganglia) compared with wild-type mice
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• Schwann cells fail to fully differentiate to a myelinating phenotype unlike in wild-type mice
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• thin and hypomyelinated
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• glia-restricted progenitors from the gut and sympathetic ganglia exhibit reduced proliferation compared with wild-type cells
• however, glia-restricted progenitors exhibit normal apoptosis
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• of peripheral nerves
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growth/size/body
• at P1
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• at P1 and P12
• at P14, mice weight one-third of wild-type mice
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• severe after birth
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limbs/digits/tail
• arthrogryposis-like paw
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