mortality/aging
• most mice die by 5 weeks of age
• all mice die by 7 weeks of age
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nervous system
• increased in the sciatic nerve
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• increased in the sciatic nerve
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• as determined by marker expression, Schwann cells do not progress beyond the immature stage unlike wild-type cells
• between P8 and P32, the number of Schwann cells in the sciatic nerve is increased slightly compared to in wild-type mice
• Schwann cells contain high amounts of rough endoplasmic reticulum compared to in wild-type mice
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• sciatic nerves are translucent, less well defined, lacks myelination, and lacks the characteristic banded pattern compared with sciatic nerves from wild-type mice
• the number of cells in the sciatic nerve is increased compared to in wild-type mice
• between P8 and P32, the number of Schwann cells in the sciatic nerve is increased slightly compared to in wild-type mice
• from P16 to P32, sciatic nerves contain increased endothelial cells, pericytes, macrophages, T lymphocytes, perineurial cells, endoneurial fibroblast, and adipocytes compared in wild-type mice
• sciatic nerves contain a dense layer of collagen and elastic fibers unlike in wild-type mice
• at P16 and P32, Remak bundles within the sciatic nerve are absent unlike in wild-type mice
• the perineural sheath is thinner than in wild-type mice
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• in the sciatic nerve
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• sciatic nerves compound action potentials lack the fast component unlike in wild-type mice
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• in the sciatic nerve
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behavior/neurological
• mice develop a straddled position of the hindlimbs unlike wild-type mice
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• mice exhibit progressive deterioration of locomotion unlike wild-type mice
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• staggering gait
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growth/size/body
• beginning at P8
• 52% lighter at P24
• 68% lighter at P32
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• mice fail to thrive shortly after birth
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cellular
• increased in the sciatic nerve
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• increased in the sciatic nerve
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