nervous system
• mice exhibit numerous axonal lesions in the corticospinal tract, especially in the major lateral tracts, unlike in wild-type mice
• axonal lesions are present in the lateral and ventral tracts at early ages and display Wallerian-like morphology unlike in wild-type mice
• in older mice, axons in the lumbar tracts are swollen unlike in wild-type mice
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• at early ages, mice exhibit axonal lesions and in older mice the area of disordered structure becomes progressively larger unlike in wild-type mice
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• disruption of the endoplasmic reticulum
• vacuolation of nerve cell bodies
• abnormal reticular aggregates
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• at P18 to P19, mice exhibit axonal lesions in the gracile nucleus in the medulla oblongata unlike wild-type mice
• by 3 months, the axonal lesions are at least twice as numerous
• degenerative axonal lesion consist of dark-staining axons with dense bodies comprising multilamellar figures or ovoids, translucent intra-axonal vacuoles and cytoskeletal elements
• mice exhibit swollen axons that progressively increase in size
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• hippocampal neuron degeneration
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behavior/neurological
• impaired performance on rotarod at 6 months of age
• when tested at 1 month of age, performance was similar to wild-type
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• mice exhibit progressive hind limb dysfunction including clenching of hind limb digits, inability to support lower body in a been walking test at 4 months, and abnormal movement in an open field test at 14 months unlike wild-type mice
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homeostasis/metabolism
• at 1 to 3 months and 6 to 12 months, neural phosphatidylcholine levels are increased compared to in wild-type mice
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growth/size/body