mortality/aging
• approximately 68% of homozygotes die before birth; surviving homozygotes are viable and fertile
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cardiovascular system
• reduced cerebral vascularization with hemorrhaging during embryonic development
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• histology of cerebral arteries from E13.5 homozygotes shows disrupted smooth muscle actin and hypoplasia
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• histology of cerebral arteries of 5 week old homozygotes shows smooth muscle cell hyperplasia
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• E13.5 cerebral vascular smooth muscle cells appear larger than normal
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hemorrhage
(
J:100236
)
• varying degrees of embryonic hemorrhaging, often associated with decreased cerebral vascularization
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• cerebrovascular hemorrhage and other vascular trauma found in E10.5-E14.5 embryos
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muscle
• histology of cerebral arteries from E13.5 homozygotes shows disrupted smooth muscle actin and hypoplasia
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• histology of cerebral arteries of 5 week old homozygotes shows smooth muscle cell hyperplasia
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• E13.5 cerebral vascular smooth muscle cells appear larger than normal
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• at 5 weeks of age, centrally located nuclei are found in approximately 17% of muscle fibers
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nervous system
• cerebrovascular hemorrhage and other vascular trauma found in E10.5-E14.5 embryos
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