Analysis Tools
cellular
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• fewer mesangial cells are present in most glomeruli
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mortality/aging
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• at 1 day after birth litter sizes from homozygous crosses are smaller; however, crosses of homozygotes to wild-type mice produce normal sized litters
• some neonates develop severe respiratory distress and die shortly after birth
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• in mice backcrossed for 5 generations to C57BL/6 the frequency of homozygotes is reduced to 16.8% by 2 weeks after birth rather than the expected 25%
• mice (backcrossed for 5 generations to C57BL/6) that die usually die within 2 weeks with most lost in the first 2 days
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cardiovascular system
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• dilated, tortuous, and poorly organized blood vessels are found in neonatal skin
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• microvessel outgrowth from cultured aortic rings is not suppressed at pH 6.9 unlike in aortic rings from wild-type controls
• however, microvessel outgrowth at pH 7.5 is similar to controls
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• dilated small arteries and veins are poorly enveloped by smooth muscle cells
• however, smooth muscle cell coverage of large vessels is similar to wild-type controls
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hemorrhage
(
J:118276
)
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• macroscopic superficial hemorrhages in the limbs, head and regions of the body wall are found in 11 of 66 embryos
• however, bleeding times and platelet numbers are similar to wild-type controls
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• hemorrhages in the parenchyma of the lung are seen in some neonates
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• seen in mice that die perinatally
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• hemorrhages in the glomeruli and parenchyma of the kidney are seen in some neonates
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• glomerular hemorrhage is seen in some neonates
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renal/urinary system
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• hemorrhages in the glomeruli and parenchyma of the kidney are seen in some neonates
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• glomerular hemorrhage is seen in some neonates
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• defect in the close contact between mesangial cells and glomerular capillaries
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• fewer mesangial cells are present in most glomeruli
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respiratory system
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• hemorrhages in the parenchyma of the lung are seen in some neonates
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• neonates displaying respiratory distress have regions of the bronchiolar epithelium with abnormal cells that have enlarged clear cytoplasm and form multiple cell layers
• this airway lining metaplasia extends peripherally to respiratory and terminal bronchioles and varies in severity
• these abnormal cells are not detected in surviving mice
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• severe respiratory distress is seen in some neonates
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homeostasis/metabolism
muscle
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• dilated small arteries and veins are poorly enveloped by smooth muscle cells
• however, smooth muscle cell coverage of large vessels is similar to wild-type controls
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integument
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• dilated, tortuous, and poorly organized blood vessels are found in neonatal skin
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