Analysis Tools
mortality/aging
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• most pups die prior to weaning at P21
(J:224155)
• most mice die before weaning
(J:241490)
• however, a minority of mice survive beyond weaning
(J:241490)
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• slightly fewer than mice are born
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nervous system
| N |
• mice exhibit normal cerebellum
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• distribution of Gfap+ radial glial cells in the dentate gyrus is slightly disturbed at E16.5 but becomes moderately and completely disorganized at P0 and P10, respectively
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• significantly fewer Gfap+ radial glial cells are found in the granular cell layer of the dentate gyrus at P10
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• at P10, Tbr2+ intermediate neuronal progenitors fail to translocate to the subgranular zone and the hilum of the dentate gyrus, with some of Tbr2+ progenitors remaining at the outer rim of the molecular cell layer
• only a few NeuroD1+ neuroblasts are present in the granular cell of the dentate gyrus at P10, and almost none is found in the subgranular zone at P24, indicating abnormal migration of NeuroD1+ neuroblasts
• cell cycle progression of the dentate migration stream is impaired at E15.5, most likely through the G1 phase
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• at P10 and P24, Ki67+ neuronal precursors are missing in the dentate gyrus, indicating a dramatic lack of proliferation in the subgranular zone
(J:224155)
• at E15.5, the number of BrdU+ progenitors is normal in the dentate migration stream whereas the Ki67+ cycling cell population (at G1, S, G2 and M, but not G0) is significantly increased, thereby decreasing the ratio of S-phase (BrdU+) over proliferating (Ki67+) cells
(J:224155)
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• at E15.5
• thinner layers V to VI at P0
• immediately after birth
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• absence of ventral and dorsal but not middle transverse sections at P14 and P24, but not E17.5 with only caudal reduction at P0
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• at P19, the hippocampus exhibits abnormal axon and dendritic trees in the dentate gyrus as well as in the CA regions
• at P10, the hippocampus fissure is malformed
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• mice exhibit partial agenesis of the hippocampus
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• at P0, the suprapyramidal blade is underdeveloped, with one end remaining attached to the ventricular zone, whereas the infrapyramidal blade is missing in the developing dentate gyrus
• at P0, a much smaller population of Ctip2+ neurons is observed in the suprapyramidal blade
• at P19, fewer neurons are observed in the dentate gyrus
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• mice exhibit dentate gyrus hypoplasia at E17.5 and P0
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• at P8, mossy fibers of the suprapyramidal bundles and dentate hilum are missing
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• at P10 and P24, the pyramidal layers of CA1 and CA3 are not as tightly packed as in control mice
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• at P19, the hippocampus exhibits disorganized neurons with less robust dendritic trees
• at P24, FoxG1+ neurons are abnormally found in the CA3 field and are not as tightly packed in the CA1 field
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• nuclear layers of CA1 and CA3 appear more diffusely packed than in control mice
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• border between cornu ammonis 1 (CA1) and the subiculum is not clearly defined at P10 or P24
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• subiculum is expanded at P10 and P24
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• altered lamination observed as early as E14.5
(J:241490)
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• shorter rostral-caudal length and thinner than in control mice
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• at P0, the population of Sox2+ neural stem cells is decreased in the dentate gyrus; no enrichment of Sox2+ neural stem cells is noted in the subgranular zone at P10 and P14, unlike in control mice
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• fewer Dcx+ immature neurons are found in the subgranular zone of the dentate gyrus at P10 and P24
• only a few NeuroD1+ neuroblasts are present in the granular cell of the dentate gyrus at P10, and almost none is found in the subgranular zone at P24
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behavior/neurological
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• one surviving mouse exhibited impaired marble burying behavior
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• impaired nest building
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growth/size/body
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• at P10, but not P5, and persisting beyond weaning
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cellular
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• reduced histone H3K23 acetylation in cortex extracts
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• cell cycle progression of the dentate migration stream is impaired at E15.5, most likely through the G1 phase; defects at E15.5 are smaller than those at or after P10
• M phase of the cell cycle appears unaffected
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• distribution of Gfap+ radial glial cells in the dentate gyrus is slightly disturbed at E16.5 but becomes moderately and completely disorganized at P0 and P10, respectively
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• significantly fewer Gfap+ radial glial cells are found in the granular cell layer of the dentate gyrus at P10
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• at P10, Tbr2+ intermediate neuronal progenitors fail to translocate to the subgranular zone and the hilum of the dentate gyrus, with some of Tbr2+ progenitors remaining at the outer rim of the molecular cell layer
• only a few NeuroD1+ neuroblasts are present in the granular cell of the dentate gyrus at P10, and almost none is found in the subgranular zone at P24, indicating abnormal migration of NeuroD1+ neuroblasts
• cell cycle progression of the dentate migration stream is impaired at E15.5, most likely through the G1 phase
|
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• at P10 and P24, Ki67+ neuronal precursors are missing in the dentate gyrus, indicating a dramatic lack of proliferation in the subgranular zone
(J:224155)
• at E15.5, the number of BrdU+ progenitors is normal in the dentate migration stream whereas the Ki67+ cycling cell population (at G1, S, G2 and M, but not G0) is significantly increased, thereby decreasing the ratio of S-phase (BrdU+) over proliferating (Ki67+) cells
(J:224155)
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| syndromic intellectual disability | DOID:0050888 | J:240552 | ||
