nervous system
N |
• despite neuron loss, no neurofibrillary tangles or plaques are observed
|
• mice exhibit a loss of white matter axons in the cerebellum
|
• mice exhibit a loss of granular neurons in the cerebellum
|
• the molecular layer in the cerebellum is atrophic
|
• mice exhibit spongiform degradation in astrocytes ranging from swelling of astrocytic foot processes to vacuolar changes in the entire cell
|
astrocytosis
(
J:125549
)
• moderate
|
• mice exhibit neuron loss accompanied by disorganization of remaining neurons with degenerative changes in their axon lengths
|
• normal axoplasm is replaced with axonal swelling with focal accumulation of organelles and often progresses to collapse of the axon
|
• dendritic processes in the CA1 region of the hippocampus exhibit collapse, vacuolization and reduction in complexity
• Purkinje dendritic processes are tortuous, dilated and exhibit reduced branching
|
• dendritic processes are tortuous, dilated and exhibit reduced branching
|
• neuronal damage
|
• mice exhibit spongiform lesions particularly in the cerebellum where loss of white matter axons, Purkinje cells and granular neurons is observed
• mice exhibit spongiform degradation in astrocytes ranging from swelling of astrocytic foot processes to vacuolar changes in the entire cell
|
behavior/neurological
• mice exhibit less severe neurological defects than observed in Tg(Gfap-Il6)G16Lms founder mouse by 1 month of age
|
paraparesis
(
J:125549
)
• hindlimb weakness
|
cardiovascular system
• marked in the cerebellum
|
growth/size/body
• at 1 to 2 weeks of age
|