mortality/aging
• under represented by 75% at weaning but found at the expected frequency at E18.5
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cardiovascular system
• cardiomegaly occurs postnatally as heart to body mass ratio is similar to controls at E18.5
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• enlarged at E18.5
• valve cusps appear hyperplastic with a 43% increase in the median number of cells and 57% increase in thickness
• increase in thickness is persistent in adults
• however, unlike in global null mice atrioventricular valve leaflets are not overtly abnormal
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• the glycosaminoglycan/proteoglycan-rich spongiosa layer of the extracellular matrix is enlarged with increased space between the granules
• 9 of 13 mutants have at least one cusp with aberrant localization and/or excessive levels of collagen
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• in surviving adults
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• at E18.5
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• increase in left ventricular end diastolic dimension in females at 24 to 28 weeks of age
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• increase in heart size is primarily due to expansion of the left ventricle
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• decrease in ejection fraction ad fractional shortening in males and females at 24 to 28 weeks of age
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• increase in aortic valve peak velocity in females at 24 to 28 weeks of age
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• at 24 to 28 weeks of age in females
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muscle
• decrease in ejection fraction ad fractional shortening in males and females at 24 to 28 weeks of age
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growth/size/body
• cardiomegaly occurs postnatally as heart to body mass ratio is similar to controls at E18.5
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