Analysis Tools
behavior/neurological
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• freeze less often than littermate controls when placed in the training environment one day after training
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• freeze less often than littermate controls when exposed to the sound cue one day after training
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• in a tube assay mice retreat more frequently compared to littermate controls
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• spend more time in the light and make more light dark transitions in a light/dark exploration assay
• spend 40% more time in the center of an open field compared to littermate controls, with no difference in the amount of movement detected
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• perform significantly better than littermate controls in most trials on a rotarod
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• 30% shorter latency to hind limb withdrawal
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• mice display seizures characterized by 6 Hz spike wave bursts with amplitudes of 150-400 uV accompanied by behavioral arrest
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• at 3.5 and 10 weeks of age, spontaneous electrographic seizures characterized by generalized attenuation of the background activity and the appearance of low-voltage fast activity, followed by generalized high frequency and high-amplitude spike and polyspike activity dissipating at different times in different brain regions are seen
• during spontaneous electrographic seizures mice generally make 4 to 10 slow versive movements of the head and/or trunk and clonic movements followed by vigorous grooming
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• at P7 the incidence of high amplitude movements including sustained spasm-like movements strong enough to cause the pup to flip over, axial contractions that bow or twist the body, and abrupt lateral displacements, is increased
• the frequency of these high amplitude movements decreases between P9 and P11
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• seen in one juvenile male
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nervous system
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• mice display seizures characterized by 6 Hz spike wave bursts with amplitudes of 150-400 uV accompanied by behavioral arrest
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• at 3.5 and 10 weeks of age, spontaneous electrographic seizures characterized by generalized attenuation of the background activity and the appearance of low-voltage fast activity, followed by generalized high frequency and high-amplitude spike and polyspike activity dissipating at different times in different brain regions are seen
• during spontaneous electrographic seizures mice generally make 4 to 10 slow versive movements of the head and/or trunk and clonic movements followed by vigorous grooming
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• at P7 the incidence of high amplitude movements including sustained spasm-like movements strong enough to cause the pup to flip over, axial contractions that bow or twist the body, and abrupt lateral displacements, is increased
• the frequency of these high amplitude movements decreases between P9 and P11
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• seen in one juvenile male
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• decrease in the number of ARX+ GABAergic interneurons in layers I-VI of the somatosensory and motor cortex
• the number of ARX+ neurons is decreased by about 50% in the hilar region of the dentate gyrus in the hippocampus and in the striatum
• all subtypes of ARX+ GABAergic interneurons are reduced except for the parvalbumin- and calretinin-expressing subtypes
• most striking loss is of the calbindin+ interneurons and this loss is seen in multiple regions
• loss of NPY+ interneurons is confined to the striatum
• severe lack of striatal cholinergic interneurons
• however, no decrease in the number of ARX+ GABAergic interneurons is seen in the parietal cortex
• fewer ARX+ interneurons in the cortex have ARX localized in the nucleus
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• the number of ARX+ neurons is decreased by about 50%
• severe lack of striatal cholinergic interneurons
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• the number of ARX+ neurons is decreased by about 50%
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• decrease in the number of ARX+ GABAergic interneurons in layers I-VI of the motor cortex and fewer ARX+ interneurons have ARX localized in the nucleus
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• decrease in the number of ARX+ GABAergic interneurons in layers I-VI and fewer ARX+ interneurons have ARX localized in the nucleus
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• at P16-20,pups display a brief myoclonic jerk that precedes a high-voltage slow transient followed by attenuation of the background EEG amplitude and a transient increase of higher frequency activity
• high-amplitude cortical spikes and sharp waves occur frequently with high voltage slow waves associated with the spike or sharp component
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endocrine/exocrine glands
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• unlike null mice, no abnormalities in pancreatic morphology or physiology are detected
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muscle
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• at P7 the incidence of high amplitude movements including sustained spasm-like movements strong enough to cause the pup to flip over, axial contractions that bow or twist the body, and abrupt lateral displacements, is increased
• the frequency of these high amplitude movements decreases between P9 and P11
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| early infantile epileptic encephalopathy | DOID:0050709 | J:151082 | ||
