growth/size/body
• males weigh marginally, but significantly, less at P30, P40, and P50 compared to wild-type males, however this is a characteristic of the nestin-cre driver line
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behavior/neurological
• mice show impaired spatial learning and memory assessed by novel object-recognition tests
• however, spontaneous alternation in Y-maze test is similar to controls
• however, 10-week-old mice show comparable exploratory behavior in the open-field test, and sociability and social novelty recognition memory in the three-chamber test as controls
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• mice show impaired retention of spatial reference memory, showing worse accuracy of spatial memory in the second probe test of the Barnes maze than in controls
• however, mice show normal acquisition of spatial reference memory in the Barnes maze test
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• mice show reduced anxiety-like behavior, spending increased time in the open arms with a similar number of entries into each arm in the elevated plus-maze
• however, time spent in the light compartment in the light-dark test is normal
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• mice exhibit longer front/hind paw overlap length, despite normal limb strength
• however, the mean stride length, stance length, and sway length of forelimbs and hindlimbs are normal
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nervous system
• density of VGLUT1 and PSD-95 puncta is decreased in a subset of hippocampal CA1 layers, the stratum oriens and stratum radiatum
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• mice show impaired excitatory synaptic development, with a reduction in the density of excitatory postsynaptic puncta
• however, neither the density nor size of inhibitory synaptic puncta are changed in neurons and gross morphology, cortical thickness and neuron numbers, including interneuron numbers, and ventricle volumes are similar to controls
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• frequency, but not amplitude, decay time, or rise slope, of miniature excitatory postsynaptic currents (mEPSCs) in cultured hippocampal neurons is decrease
• however, the frequency or amplitude of miniature inhibitory postsynaptic currents (mIPSCs) is unaffected
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
non-syndromic X-linked intellectual developmental disorder 111 | DOID:0060929 |
OMIM:301107 |
J:342291 |