Analysis Tools
behavior/neurological
 |
• in the object recognition test, no difference in short-term recognition memory is seen at P40, however the discrimination index at 24 hours is lower, indicating that capacity to recall a familiar object is impaired
|
|
|
• this memory deficit becomes more pronounced at P100 and eventually affects both short- and long-term memories at P180, such that mice show a memory deficit both at 1- and 24-hour interval
|
|
|
• this memory deficit becomes more pronounced at P100 and eventually affects both short- and long-term memories at P180, such that mice show a memory deficit both at 1- and 24-hour interval
|
|
|
• in the Morris water maze, mice show a spatial memory impairment in the probe test with mice spending less time in the quadrant where the platform was located during training and equally exploring the four quadrants of the maze, indicating they dont remember the location of the hidden platform
• progression of cognitive deficits is seen in the training phase of Morris water maze, with mice at P180
• mice at P180 show similar learning and memory in the Morris water maze as 1 year old wild-type mice
|
|
|
• in the Y maze spontaneous alternation, mice show a lower alternation rate than controls at P28, P40, P100 and P180
|
|
|
• mice at P180, but not P40, spend about 3-fold more time grooming than wild-type mice
• however, mice show normal anxiety responses at P40, P100, and P180 and normal behavior in the social preference test and the social novelty task
|
|
|
• mice show increase of fall latency form the rotarod at P40, P100 and P180
|
cellular
|
|
• number of Ki67-positive cells in the dentate gyrus is reduced about 30%, indicating reduced proliferation/neurogenesis
|
growth/size/body
|
|
• reduction in body weight at P60, P100, and P180
|
hematopoietic system
microgliosis
(
J:238578
)
|
|
• increase in activated microglial cells in the brain with a parallel reduction of resting cells
|
homeostasis/metabolism
|
|
• creatine levels in the brain, muscle, heart and kidney are reduced in 1 month and 6 month old mice; this decrease is the same at P30 as in P180 mice, except in the muscle which show s greater reduction at P180 than at P30
• moderate increase in guanidinoacetic acid levels in the brain, muscle and heart at P30 and in the brain and muscle at P180
|
immune system
microgliosis
(
J:238578
)
|
|
• increase in activated microglial cells in the brain with a parallel reduction of resting cells
|
nervous system
|
|
• number of Ki67-positive cells in the dentate gyrus is reduced about 30%, indicating reduced proliferation/neurogenesis
|
microgliosis
(
J:238578
)
|
|
• increase in activated microglial cells in the brain with a parallel reduction of resting cells
|
|
|
• number of Ki67-positive cells in the dentate gyrus is reduced about 30%, indicating reduced proliferation/neurogenesis
• doublecortin-positive cells are reduced in the dentate gyrus, indicating reduced number of immature neurons at P180
|
|
|
• hippocampal volume is reduced at P180
|
|
|
• mice show loss of GABAergic synapses in the cerebral cortex
|
|
|
• mice show loss of GABAergic synapses in the cerebral cortex
• however, excitatory synapses are not affected
|
pigmentation
|
|
• accumulation of lipofuscin throughout the brain, especially in the dentate gyrus hippocampal region at P180
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| cerebral creatine deficiency syndrome 1 | DOID:0050800 |
OMIM:300352 |
J:238578 | |
