Analysis Tools
mortality/aging
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• die by 22 days of age
(J:81516)
• mice die between P1 and P21
(J:142703)
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nervous system
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• often by 1 week of age and usually severe with lateral ventricles most severely affected, the third ventricle less severely affected, and the fourth ventricle normal
(J:81516)
• severe at P16
(J:142703)
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• enlarged
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• astrocytic hypertrophy and hyperplasia in brain stem and spinal cord and to a much lesser extent in the cerebral cortex, thalamus, and midbrain, within the first weeks of life with most homozygotes developing hydrocephalus and a few developing status spongiosis
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• astrocytes from affected mice cease dividing shortly after being put in culture
(J:81516)
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• in several regions of the brain including thalamus, brain stem and cerebellar nucleus
(J:142703)
• however, spinal motor neurons and sensory neurons remain intact
(J:142703)
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behavior/neurological
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• at P14, mice exhibit impaired motor function
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growth/size/body
pigmentation
integument
