nervous system
• accumulation of myelin debris in Schwann cells, increase in the number of mast cells and defects in unmyelinated Schwann cells
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• accumulation of lipid-containing particles in the perineurial and endoneurial compartments
• however, unlike Lpin1fld homozygotes no reduction is seen in the number of adipocytes in the epineurium
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• strong demyelination of the sciatic nerve similar to Lpin1fld homozygotes
• accumulation of myelin debris in Schwann cells, increase in the number of mast cells and defects in unmyelinated Schwann cells
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• reduced motor nerve conduction velocity is accompanied by temporal dispersion and reduction of compound muscle action potentials
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• strong reduction in motor nerve conduction velocity of the sciatic nerve at P56
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behavior/neurological
• clench toes of the hind feet when lifted by the tail
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• develops by 2 weeks of age
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