Analysis Tools
mortality/aging
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• about 6% of mice die of hydroencephaly by 3 weeks of age
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nervous system
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• some facial neurons migrate into the fourth ventricle and form a single group of cells in contrast to wild-type mice, where facial neurons migrate from their origin in the neuroepithelium on each side of the neural tube to form two separate groups on the left and right side of the brain stem
• some mutant neurons reach their intended destination, but number is much lower than in wild-type mice (96 neurons for mutants vs 208 for wild-type)
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hydrocephaly
(
J:112536
)
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• ~6% of homoyzgotes die by 3 weeks of age due to progressive hydrocephalus; upon necropsy, all homozygotes show evidence of hydrocephalus
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• some migrating facial neurons in mutants protrude into the ventricle, whereas wild-type neurons migrate more posteriorly; correlates with hydroencephaly
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• cerebral ventricles are extensively dilated including the lateral and third ventricles
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• commissural fibers abnormally cross through the aqueduct, disrupting the ependymal layer
• the lumen of the aqueduct is completely blocked
• defects in the aqueduct of the Sylvius are sufficient to result in a progressive hydrocephalus in 6% of mice
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cardiovascular system
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• cardiac myocytes have an average area that is 2 times larger than control
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cellular
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• some facial neurons migrate into the fourth ventricle and form a single group of cells in contrast to wild-type mice, where facial neurons migrate from their origin in the neuroepithelium on each side of the neural tube to form two separate groups on the left and right side of the brain stem
• some mutant neurons reach their intended destination, but number is much lower than in wild-type mice (96 neurons for mutants vs 208 for wild-type)
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