Analysis Tools
mortality/aging
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• most die during birth with only 10% surviving to P1 and none surviving to P2
• however, no loss of mice is seen from E11.5 to E18.5
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• homozygous embryos die around E12.5, with embryos appearing partially abnormal and/or partially resorbed by E12.5
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cardiovascular system
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• tortuous with variable irregularities including narrowing dilation, aneurysms, and ruptures
• defects are most severe in the aorta and other large arteries but occur in others as well
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• at E15.5 the aorta is noticeably tortuous and irregular and this becomes more severe with age
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• at E12.5 the outer diameter is reduced to 1/2 to 2/3 of wild-type; however, the number of cells in the wall is not increased compared to wild-type
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• no continuous elatic lamina is seen at any age from E12.5 to P1
(J:106902)
• at E14.5 irregular elastin aggregates are seen and these become larger and more numerous with age
(J:106902)
• at P1 decreased desmosine levels indicate a 94% decrease in elastin cross linking
(J:106902)
• the aortic elastic laminae is complete disarranged unlike in wild-type mice
(J:154659)
• the elastogenesis is abolished unlike in wild-type mice
(J:154659)
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• at E12.5 aortic smooth muscle cells appear rounder and less stretched although this becomes less obvious at E13.5 and later ages
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• the walls are about twice as thick as in wild-type mice
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• uniform narrowing (stenosis) of the descending aorta in mutant embryos at embryonic day 12.5 (E12.5)
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hemorrhage
(
J:106902
)
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• resulting from ruptured arteries
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respiratory system
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• at P1 no fine elastic fibers are detected and decreased desmosine levels indicate an 88% decrease in elastin cross linking
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muscle
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• at E12.5 aortic smooth muscle cells appear rounder and less stretched although this becomes less obvious at E13.5 and later ages
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integument
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• at P1 no fine elastic fibers are detected in the hypodermal connective tissue; however, no gross changes in skin condition are seen
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