nervous system
• cerebellum is smaller compared to wild-type
• abnormalities in cerebellar lamination are observed; clusters or single ectopic granule cells are seen in the molecular layer and white matter with single cells most frequent in the vermis and in the hemisphere
|
• pattern of foliation in mutants is variable; changes in foliation differ in the anterior vs posterior cerebellum
• in some mice, the size of the posterior cerebellum is decreased and the anterior cerebellum is increased in size; in others, the posterior cerebellum is relatively enlarged accompanied by relative anterior cerebellum size decrease
• in the anterior-most cerebellum there is a discontinuity between lobes I-II and lobe III with the pial surface in this region apparently in direct contact with the underlying white matter
• small microfolia are present in homozygotes; these are much smaller and many are pointed rather than rounded at the cerebellar surface
|
• the granule cell layer is thinner compared to wild-type and granule cells appear to be smaller and lower in number; ectopic granule cells are seen along the entire extent of the molecular layer with greatest numbers above the tip of the suprapyramidal limb
• dark cells in the deep granule cell layer that are seen in wild-type are only rarely seen in homozygotes
|
• packing density of pyramidal cell layer is significantly decreased
|
• pyramidal cells appear smaller than in wild-type
|
• in layer CA2 there appears to be a decrease in neuron number
|
• granule and molecular layers are markedly thinner in the wall facing the fissure and thicker in the wall facing the adjacent normal folium; Purkinje cells are absent from the the thinner wall and in the wall facing the normal folium, Purkinje cells are fewer and smaller
|
• in some mice, clusters of granule cells are situated ectopically in the white matter of lobes I-II and lobe III with lobes IV-V merged into a single large folium
|