Analysis Tools
behavior/neurological
| N |
• no differences are seen in the light-dark exploration test between mutants and wild-type mice
• mutants treated with the drug Risperidone exhibit rescue of the hyperactivity, repetitive behavior/perseveration and nesting deficits
|
|
• mutants perform similarly to wild-type mice in the Morris water maze probe trials, however in a classic reversal task using the Morris water maze, mutants show impaired learning of the new location of the platform and perform poorly in the probe test
|
|
• mutants spend almost 3 times more time grooming than wild-type mice
|
|
• mutants perform better than wild-type mice on the rotorod
|
|
• mutants show greater locomotor activity than wild-type mice in the open field test
• mutants treated with the drug Risperidone exhibit rescue of the hyperactivity
|
|
• in the spontaneous alternation T maze test, mutants show higher number of no alternations in a standard 10 trial test, indicating preservation
• mutants treated with the drug Risperidone exhibit rescue of the repetitive behavior/perseveration
|
|
• mutants exhibit hyperreactivity to thermal sensory stimuli
• however, no differences seen in the acoustic startle response or prepulse inhibition
|
|
• mutants are impaired in nest-building behavior, scoring less than half of the wild-type criterion
• mutants treated with the drug Risperidone exhibit rescue of the nesting deficits
|
|
• in a juvenile play test, mutants at P21 spend less time interacting with each other and instead show increased repetitive behaviors such as grooming and digging
• in a three-chamber social interaction test in adults, mutants do not show a preference for the cup with a mouse as seen in wild-type mice
|
|
• mutants emit a lower number of ultrasonic calls than wild-type mice at all ages
|
|
• seizures are induced by mild stressors during routine handling
|
nervous system
|
• seizures are induced by mild stressors during routine handling
|
|
• mutants exhibit a reduction in parvalbumin+ interneurons in the hippocampus
• mutants exhibit a decrease in the number of GABAergic interneurons in all laminae and in the striatum
|
|
• ectopic neurons are seen in the corpus callosum of mutants at P14; ectopic neurons are seen through adulthood
|
astrocytosis
(
J:177110
)
|
• reactive astrocytosis is seen throughout the hippocampus after onset of seizures, especially in the hilus
• however, neuronal loss is not seen in the hippocampus
|
|
• at 8 months of age, freely moving mutants exhibit generalized interictal spike discharges during slow-wave sleep
|
|
• mutants exhibit reduced cortical neuronal synchrony as indicated by two-photon calcium imaging of layer II/III neurons from somatosensory cortex showing that neuronal firing pattern is highly asynchronous compared to wild-type mice
• however, neither the average firing amplitude nor the average firing rate are changed, suggesting that the defect is due to a network dysfunction rather than abnormalities in neuronal activity or conduction per se
|
|
• ectopic neurons are seen in the corpus callosum of mutants at P14
• mutants exhibit abnormal migration of cortical projection neurons as evidenced by the higher numbers of CUX1+ cells in deep cortical layers
|
taste/olfaction
|
• mutants perform better than wild-type mice in the buried food test, indicating better olfaction
|
cellular
|
• ectopic neurons are seen in the corpus callosum of mutants at P14
• mutants exhibit abnormal migration of cortical projection neurons as evidenced by the higher numbers of CUX1+ cells in deep cortical layers
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autism spectrum disorder | DOID:0060041 | J:177110 | ||
| cortical dysplasia-focal epilepsy syndrome | DOID:0090130 |
OMIM:610042 |
J:177110 | |
