behavior/neurological
• hopping and trembling
|
embryo
• disorganized ependymal cells lining ventral caudal 3rd ventricle
• absent ependymal cells lining ventral caudal 3rd ventricle in areas of ventricle closure
• undulated appearance: distorted overall outline of ependymal cells marked by adherens junctions, with bent and strained cell-cell boundaries, first observed at P0.5
• single layer of ependymal cells transformed into multilayer configuration at multiple sites
• disordered mature ependymal epithelial cells form amorphous accumulations of cells obstructing ventricular passage
• significant reduction in number of mature multiciliated ependymal cells in ventral caudal 3rd ventricle
|
growth/size/body
nervous system
N |
• subarachnoid space and 4th ventricle morphology
|
• disorganized ependymal cells lining ventral caudal 3rd ventricle
• absent ependymal cells lining ventral caudal 3rd ventricle in areas of ventricle closure
• undulated appearance: distorted overall outline of ependymal cells marked by adherens junctions, with bent and strained cell-cell boundaries, first observed at P0.5
• single layer of ependymal cells transformed into multilayer configuration at multiple sites
• disordered mature ependymal epithelial cells form amorphous accumulations of cells obstructing ventricular passage
• significant reduction in number of mature multiciliated ependymal cells in ventral caudal 3rd ventricle
|
• postnatal
|
• highly dilated
|
• distorted and closed ventral caudal 3rd ventricle
|
• highly dilated rostral to closed region of aqueduct
|
• at ventral caudal 3rd ventricle
• disordered mature ependymal epithelial cells form amorphous accumulations of cells obstructing ventricular passage
|
• generally asymmetric and distorted bilobed canal at dorsal side of rostral aqueduct
• partially closed, forming two separate small lumens lined by ependymal epithelial cells
• discontinuities with denudations of ependymal epithelial layer in median aqueduct, especially in vaults
• undulated appearance: distorted overall outline of ependymal cells marked by adherens junctions, with bent and strained cell-cell boundaries, first observed at P0.5
• single layer of ependymal cells transformed into multilayer configuration at multiple sites
• becomes progressively narrower and ultimately closes (stenosis) between P0.5 and P14.5
• significant reduction in number of mature multiciliated ependymal cells in rostral aqueduct
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
obstructive hydrocephalus | DOID:14159 | J:251454 |