Analysis Tools
mortality/aging
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• homozygotes die by E10.5, as a result of heart failure
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cardiovascular system
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• homozygotes exhibit a normal initial assembly of I-Z-I complexes into nascent myofibrils with overlapping actin filaments and regularly spaced dots of sarcomeric alpha-actinin
• in addition, initial assembly of thick filaments, as monitored by myomesin, appears unaffected
• however, nascent myofibrils never mature into striated myofibrils and gaps at the H zone are never observed; instead, thin filaments and myofibrils appear unstable, forming aberrant aggregates at both E8.5 and E9.5
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• at E9.5, homozygotes lack well-defined myocardial trabeculations
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• at E9.5, homozygotes exhibit an abnormally thin myocardium (~2-cell layers in thickness)
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• at E8-E8.5, homozygotes exhibit a normal early myocardium and matrix in the heart tube
• however, at E9.5, all mutant hearts exhibit failure of myofibril assembly resulting in aborted myocardial development with variable severity; as a result, cardiac development resembles that of E8-E8.5 embryos
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• at E9.5, homozygotes show complete lack of cellularization within the AV canal
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• by E10.5, homozygotes display a distended pericardial cavity containing the malformed heart
• pericardial distension is attributed to fluid leakage into the pericardium
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• at E9.5, mutant hearts fail to pump; ink injected into mutant embryos remains within the heart tube, dispersing by passive diffusion over 6 hrs in culture
• as a result, pooling of erythrocytes is frequently observed in the malformed heart cavity
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• at E9.5, mutant hearts lack a functional contractile activity
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• in culture, hearts from E9.5 mutant embryos fail to beat and only twitch once or twice during the first 15-20 min after isolation and in response to initial contact with the microinjection needle
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embryo
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• homozygotes fail to grow beyond E9.5
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• by E9.5, mutant embryos are significantly malformed and smaller than wild-type
• however, at E8-E8.5 (before embryo turning), homozygotes exhibit normal size and morphology
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• by E9-E9.5, the mutant yolk sac appears very wrinkled and folded
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growth/size/body
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• by E9.5, mutant embryos are significantly malformed and smaller than wild-type
• however, at E8-E8.5 (before embryo turning), homozygotes exhibit normal size and morphology
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• by E9.5, homozygotes display obvious defects in development of the head structures
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muscle
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• homozygotes exhibit a normal initial assembly of I-Z-I complexes into nascent myofibrils with overlapping actin filaments and regularly spaced dots of sarcomeric alpha-actinin
• in addition, initial assembly of thick filaments, as monitored by myomesin, appears unaffected
• however, nascent myofibrils never mature into striated myofibrils and gaps at the H zone are never observed; instead, thin filaments and myofibrils appear unstable, forming aberrant aggregates at both E8.5 and E9.5
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• at E9.5, homozygotes lack well-defined myocardial trabeculations
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• at E9.5, homozygotes exhibit an abnormally thin myocardium (~2-cell layers in thickness)
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• at E9.5, mutant hearts lack a functional contractile activity
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• homozygotes display abnormal formation of regulated actin filament lengths, as well as absence of myofibril maturation and sarcomeric integrity during de novo myofibril assembly in the developing heart
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