Analysis Tools
nervous system
| N |
• at 6 weeks of age, mice exhibit normal brain size, with no detectable gross abnormalities in brain anatomy
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• mice exhibit impaired synapse maturation in cortical layer II/III, with higher numbers of immature synapses detected in the cerebral cortex
• at P21, Stat1 expression in the cortex is significantly higher than in wild-type controls
• inhibition of STAT1 expression partially rescues impaired synaptic formation
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• at 8 weeks of age, the percentage of mushroom-type mature spines on apical dendrites of cortical layer II/III pyramidal neurons is lower than in wild-type controls
• moreover, at 6 weeks of age, the density of mushroom-type mature spines is significantly lower than in wild-type controls
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• at 6 weeks of age, the density of mushroom-type mature spines in layer II/III pyramidal neurons is significantly lower than in wild-type controls
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• at 8 weeks of age, spine length of cortical layer II/III pyramidal neurons is significantly greater than in wild-type controls
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• at 8 weeks of age, the percentage of thin- and filopodia-type immature spines on apical dendrites of cortical layer II/III pyramidal neurons is higher than in wild-type controls
• in contrast, the percentage of stubby spines is normal
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• at 3 and 6 weeks of age, cortical layer II/III pyramidal neurons exhibit greater dendritic arbor complexity and dendrite length than in wild-type controls
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• expression of the excitatory postsynaptic protein postsynaptic density 95 (PSD-95) is reduced, with no significant change in the level of the excitatory presynaptic protein SLC17A7 (aka VGlut1)
• at 6 weeks of age, EM analysis showed significantly thinner and shorter postsynaptic densities than in wild-type controls
• after culture for 21 days, cortical neurons transfected with control siRNA show fewer numbers of PSD-95-positive puncta than wild-type neurons; however, lower PSD-95 density is partially rescued by Stat1 siRNA transfection
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behavior/neurological
 N |
• adult males (11-week-old or older) show no significant alterations in locomotion, nociception, social behavior, motor coordination, depression-like behavior or spatial memory
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• in the light/dark transition test, adult males exhibit a significantly longer latency to first entry into the light box, suggesting increased anxiety; however, distances traveled in the light or dark boxes, stay time in the light box, and the number of transitions are not altered
• in the marble-burying test, adult males show more marble-burying behavior with no difference in total distance traveled
• in the novelty-induced hypophagia test, adult males show a significantly longer latency to drink sweetened condensed milk in a novel, brightly lit cage; however, milk consumption in the novel cage is not significantly altered
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• in a 24-h home cage-monitoring test, mice exhibit hyperactivity during the nighttime period
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growth/size/body
| N |
• at 1-8 weeks of age, both sexes exhibit normal body size, with no significant alterations in body weight relative to wild-type controls
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Cornelia de Lange syndrome 3 | DOID:0080507 |
OMIM:610759 |
J:242006 | |
