behavior/neurological
• mild motor deficits detected by an accelerating rotarod test that become prominent at 8 months of age
• performance in an accelerating rotarod test declines until about 12 months of age
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• modest decrease in performance in a muscle strength test
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nervous system
• at 18 months of age a high number of degenerating axons are found in corticospinal tracts
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• the periaxonal space, of myelinated motor axons in the sciatic nerve and CNS corticospinal tract, is frequently dilated
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• slight decrease in axon numbers in L5 ventral roots
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• at 18 months of age a high number of degenerating axons are found in corticospinal tracts
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• transport of tubulin, but not of neurofilaments, is slower compared to controls
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
NOT | amyotrophic lateral sclerosis type 2 | DOID:0060194 |
OMIM:205100 |
J:138147 |