Analysis Tools
mortality/aging
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• homozygotes obtained from crosses with 129S/SvEv mice are alive at 12 hours after birth; however, most of them die within 48 hours after birth
• no homozygotes survive to weaning
• Background Sensitivity: selective breeding of the 5% of homozygotes that survive on a mixed genetic (129P2/OlaHsd x C57BL/6 x DBA/2) background results in a 21% survival rate
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growth/size/body
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• homozygotes exhibit a higher lung-to-body-weight ratio relative to wild-type mice
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cardiovascular system
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• mutant lungs display congestion of pulmonary septal capillaries
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• in a number of homozygotes that die at >24 h after birth, mutant livers display changes compatible with passive congestion
• such changes are suggestive of acute ischemia secondary to heart failure
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• mutant lungs exhibit congestion of pulmonary septal capillaries
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• although the DA appears constricted relative to the fetal DA at 3 hrs after birth, homozygous newborns exhibit obvious patency of the DA at both 7 and 12 hrs after birth
• at 12 hrs, swelling of the intima absent and the endothelium remains a single layer of cells contiguous with that of the aorta
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• homozygotes exhibit hemorrhaging into the alveolar spaces and major respiratory ducts
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• homozygotes show a postnatal drop in pulmonary vascular resistance
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• mutant lungs display a left-to-right shunt of blood
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respiratory system
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• homozygotes exhibit hemorrhaging into the alveolar spaces and major respiratory ducts
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• mutant lungs exhibit congestion of pulmonary septal capillaries
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• homozygotes exhibit a higher lung-to-body-weight ratio relative to wild-type mice
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immune system
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• homozygotes display dilated perivascular lymphatics
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liver/biliary system
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• in a number of homozygotes that die at >24 h after birth, mutant livers display changes compatible with passive congestion
• such changes are suggestive of acute ischemia secondary to heart failure
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• in a number of homozygotes dying at >24 h after birth, mutant livers contain macrovesicular lipid deposits
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• in a number of homozygotes dying at >24 h after birth, mutant livers contain microvesicular lipid deposits
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homeostasis/metabolism
cellular
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• although the DA appears constricted relative to the fetal DA at 3 hrs after birth, homozygous newborns exhibit obvious patency of the DA at both 7 and 12 hrs after birth
• at 12 hrs, swelling of the intima absent and the endothelium remains a single layer of cells contiguous with that of the aorta
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