nervous system
• reactive microglia
• mice exhibit an increase in CD68-positive cells in cortex, thalamus and hippocampus
• cells have a macrophage-like appearance with rounded cell bodies and shortened processes in the cortex, thalamus and hippocampus
|
astrocytosis
(
J:213552
)
• mice exhibit an increase in GFAP immunoreactivity in the cortex, thalamus and hippocampus as compared to controls
• astrocytes throughout the brain appear hypertrophied with enlarged cell soma and thickened processes as compared to controls
|
pigmentation
• autofluorescent storage material is observed in the cortex, thalamus and hippocampus of 5 month old mice
|
hematopoietic system
• reactive microglia
• mice exhibit an increase in CD68-positive cells in cortex, thalamus and hippocampus
• cells have a macrophage-like appearance with rounded cell bodies and shortened processes in the cortex, thalamus and hippocampus
|
immune system
• reactive microglia
• mice exhibit an increase in CD68-positive cells in cortex, thalamus and hippocampus
• cells have a macrophage-like appearance with rounded cell bodies and shortened processes in the cortex, thalamus and hippocampus
|
behavior/neurological
• males exhibit decreased exploration, staying in a large Petri dish longer than wild-type mice at 3 and 5 months of age
|
• in a modified vertical pole test, 3 and 5 month old mutants climb down on the pole slower and turn downward on the pole slower than wild-type mice
|
• 3 month old mutants fall from the rotating rod sooner than wild-type mice, however performance is similar to wild-type mice at 5 months of age
|
growth/size/body
• mice are heavier at 3 months of age, however the weight difference is gone by 5 months of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
neuronal ceroid lipofuscinosis 1 | DOID:0110721 |
OMIM:256730 |
J:213552 |