cardiovascular system
• pulmonary arteries connect to aorta instead of the ouflow tract
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• embryos have a disorganized vascular endothelial plexus along anterior foregut which does not connect to heart inflow and outflow tracts
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• reduction in vascular and airway smooth muscle derived from Isl1- and Gli1-positive cardiopulmonary mesoderm progenitors is detected
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• pulmonary veins from left atrium to lung are absent
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• mutants show reduced muscular development in dorsal wall of atria
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endocrine/exocrine glands
• shortened cystic duct at E14.5
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• shortened gallbladder at E14.5
• however, the gallbladder epithelium is histologically normal
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• formation of smooth muscle layers in gallbladders is delayed and defective
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muscle
• reduction in vascular and airway smooth muscle derived from Isl1- and Gli1-positive cardiopulmonary mesoderm progenitors is detected
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• mutants show reduced muscular development in dorsal wall of atria
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• formation of smooth muscle layers in gallbladders is delayed and defective
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growth/size/body
liver/biliary system
• shortened cystic duct at E14.5
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• shortened gallbladder at E14.5
• however, the gallbladder epithelium is histologically normal
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• formation of smooth muscle layers in gallbladders is delayed and defective
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• fetuses exhibit severe growth retardation in the liver without signs of hepatic inflammation
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limbs/digits/tail
• a limited skeletal structure consisting of the humerus, radius and single digit 1 is seen
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skeleton
• a limited skeletal structure consisting of the humerus, radius and single digit 1 is seen
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respiratory system
• airway smooth muscle derived from Isl1- and Gli1-positive cardiopulmonary mesoderm progenitors is significantly reduced; airway smooth muscle development is reduced
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