Analysis Tools
mortality/aging
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• only very few mice survive to adulthood
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• most mice die during the weaning period
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growth/size/body
| N |
• mice do not develop sinus inversus
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• body weight is significantly lower than that in control mice after P3 to P19
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megacephaly
(
J:294184
)
|
• pups fail to grow normally
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craniofacial
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• the few mice surviving to adulthood exhibit an enlarged skull
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• the few mice surviving to adulthood exhibit an enlarged dome-shaped skull at 3 months of age
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behavior/neurological
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• the few mice surviving to adulthood exhibit apathy
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muscle
|
• the few mice surviving to adulthood exhibit muscle weakness
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nervous system
| N |
• mice exhibit no defects in neurogenesis, neuronal migration or astrocyte development relative to wild-type controls
|
|
• at P8, cerebral vessel density and branching frequency are significantly reduced in mice with hydrocephalus
|
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• at P7, ependyma cilia exhibit a lower beating amplitude and a disorderly rotation beating pattern of cilia bundles
• however, beating frequency is not significantly affected
|
microgliosis
(
J:294184
)
|
• IbaI-positive microglial cell number is markedly increased in the cerebral cortex at P8
|
|
• at P8, almost all microglia are activated as indicated by their shorter processes and larger soma
|
hydrocephaly
(
J:294184
)
|
• mice exhibit severe progressive hydrocephalus due to postnatal developmental defects
• however, no brain hemorrhage is observed
|
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• mice show enlarged brain hemispheres at P8
|
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• immunofluorescence staining with an S100beta (an ependyma marker) antibody indicates a slight rupture of the ependymal layer at P8, followed by severe damage and ependymal exfoliation at P12
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• although most ependymal cilia exhibit normal axonemes with the expected 9 + 2 ultrastructure, a few show a disorganized (6 + 1 or 5 + 0) axonemal structure; the central pair of microtubules and the outer microtubule doublets may turn into single microtubules or become vacant
• ependymal cilia frequently display abnormal ectopic ciliary membrane inclusions and the centriolar patch size is significantly increased
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• cerebral ventricular size is significantly increased at P8
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• enlarged lateral ventricles at P10
• the area of lateral cerebral ventricular zone is increased at P3 and P7
|
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• the hypothalamus is severely compressed
|
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• the hippocampus is severely compressed
|
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• severe thinning of the cerebral cortex at P10
|
astrocytosis
(
J:294184
)
|
• GFAP-staining is markedly increased in the cerebral cortex at P8, indicating astrogliosis
|
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• at P8, expression of oligodendrocyte transcription factor 2 (OLIG2, a marker of mature oligodendrocytes) is enhanced while the number of OLIG2+ cells is significantly increased in the medial ganglionic eminence
|
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• mice show massive accumulation of CSF in the lateral ventricles at P10
• at 10 min after injection of Evans blue dye into the anterior horn of the lateral ventricle, very little/no tracer can be detected in the third and fourth ventricles, unlike in wild-type controls
• however, CSF is clear with no signs of hemorrhage and no aqueduct stenosis is observed at P8
|
|
• at P8, expression of myelin basic protein (MBP, a marker of myelinating glia) is decreased, suggesting myelination defects
|
immune system
microgliosis
(
J:294184
)
|
• IbaI-positive microglial cell number is markedly increased in the cerebral cortex at P8
|
|
• at P8, almost all microglia are activated as indicated by their shorter processes and larger soma
|
|
• mice develop severe sinusitis; sinuses are filled with pus
|
cardiovascular system
|
• at P8, cerebral vessel density and branching frequency are significantly reduced in mice with hydrocephalus
|
cellular
|
• although most ependymal cilia exhibit normal axonemes with the expected 9 + 2 ultrastructure, a few show a disorganized (6 + 1 or 5 + 0) axonemal structure; the central pair of microtubules and the outer microtubule doublets may turn into single microtubules or become vacant
• ependymal cilia frequently display abnormal ectopic ciliary membrane inclusions and the centriolar patch size is significantly increased
|
|
• at P7, ependyma cilia exhibit a lower beating amplitude and a disorderly rotation beating pattern of cilia bundles
• however, beating frequency is not significantly affected
|
hematopoietic system
microgliosis
(
J:294184
)
|
• IbaI-positive microglial cell number is markedly increased in the cerebral cortex at P8
|
|
• at P8, almost all microglia are activated as indicated by their shorter processes and larger soma
|
respiratory system
|
• mice develop severe sinusitis; sinuses are filled with pus
|
skeleton
|
• the few mice surviving to adulthood exhibit an enlarged skull
|
|
• the few mice surviving to adulthood exhibit an enlarged dome-shaped skull at 3 months of age
|
