behavior/neurological
• by P60, mice have severely disrupted balance, frequently drag their bodies along the ledge and fall on their heads when lowering themselves into the cage
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• older mice exhibit locomotor defects
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• mice exhibit an aberrant halting gait and frequently lose their balance by P30 (but not a P21) in the ledge test and gait analysis
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hematopoietic system
microgliosis
(
J:282431
)
• reactive microglia become detectable in the deep cerebellar nuclei at P22 and gliosis becomes more severe at P30
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immune system
microgliosis
(
J:282431
)
• reactive microglia become detectable in the deep cerebellar nuclei at P22 and gliosis becomes more severe at P30
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nervous system
microgliosis
(
J:282431
)
• reactive microglia become detectable in the deep cerebellar nuclei at P22 and gliosis becomes more severe at P30
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• a major reduction in Purkinje cell number is seen at 10 months of age
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• by P30, Purkinje cell primary and secondary dendrites appear swollen
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• by P60, the deep cerebellar nuclei contains large aggregates throughout which are positive for both poly-Ub conjugates and p62
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astrocytosis
(
J:282431
)
• by P30, a dramatic increase in reactive astrocytes is seen in the DCN, but not the Purkinje or molecular layers
• by P50, some reactive astrocytes are seen in the Purkinje layer
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• swellings along the length of the axons in the white matter
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• Purkinje cell axon terminals in the deep cerebellar nuclei have swellings
• Purkinje cells lose almost all of their axon terminals in the deep cerebellar nuclei by 10 months of age
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• axonal torpedoes, focal swellings on Purkinje cell axons are seen by 10 months of age
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• progressive damage to Purkinje cell axon terminals
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