nervous system
N |
• mice do not exhibit neuronal death or reactive gliosis in noncerebellar region
|
• reduced microtubule network
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• at 7 months in the cerebellar cortex and by 18 months in the cerebellum
• transgene expression of the LC2 isoform doesnt not rescue Purkinje cell degeneration
• however, no loss is observed prior to 3 months and transgene expression of the precursor gene rescues Purkinje cell loss
|
• focal swellings beginning at 6 weeks and becoming more widespread with age
|
• at 18 months of age
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• axonal torpedos beginning at 6 weeks of age
|
• hypertrophic or exhibiting abnormal blebs at 2 months
• transgene expression of the LC2 isoform doesnt not rescue the axon initial segment phenotype
|
behavior/neurological