Analysis Tools
mortality/aging
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• ~25% of homozygotes die 1-2 days prior to birth (E17-E18.5) with a hemorrhagic and edematous phenotype
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• ~75% of homozygotes die at birth
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renal/urinary system
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• at E17.5 or later, homozygotes exhibit a spotty kidney phenotype
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• the mutant Bowman's capsule space contains blood-filled aneurysm-like structures
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• at E17.5 or later, homozygotes exhibit abnormal mature glomerular bodies ("red spots") in the inner part of the cortex, with capillary aneurysms replacing the glomerular tufts
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• at E17.5 or later, mutant glomeruli show absence or reduction of mesangial cells in the presence of a morphologically normal basement membrane
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• at E17.5 or later, mutant glomeruli may show absence of mesangial cells
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small kidney
(
J:20017
)
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• at E17.5 or later, homozygotes exhibit smaller kidneys
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• at E17.5 or later, homozygotes display empty urinary bladders, suggesting kidney dysfunction
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cardiovascular system
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• at E17.5 or later, homozygotes exhibit dilated thoracic large arteries as well as dilated mesenteric blood vessels
(J:20017)
• however, no hypoplasia of smooth muscle cells is observed
(J:20017)
• exhibit regional dilations along the blood vessel length that are not seen in wild-type
(J:78912)
• exhibit a 2.5-fold increase in the number of caveolae in brain vessels
(J:78912)
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• at E17.5 or later, homozygotes display dilated thoracic large arteries
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• at E16.5-E19, homozygotes display a dilated aorta with an extended, thinner aortic muscular wall
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dilated aorta
(
J:20017
)
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• at E16.5-E19, homozygotes display a dilated aorta with an extended, thinner aortic muscular wall
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• endothelial cells in the brain exhibit a luminal surface that has multiple processes projecting into the lumen of the vessel
• thickness of endothelium varies considerably; in some areas the cytoplasm of the cells is very thin and in others it is thicker than in wild-type
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• exhibit endothelial cell hyperplasia in the brain from E11.5 on, however hyperplasia is not seen in the perineural vascular plexus surrounding the brain
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• exhibit increased capillary diameter in the brain
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• the mutant Bowman's capsule space contains blood-filled aneurysm-like structures
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• exhibit loss and altered organization of pericytes in placentas; pericytes are found in the labyrinthine layer but are reduced by about 50% and are completely absent from the spongiotrophoblast layer
• pericytes are displaced from multicellular core aggregates to scattered sites in the walls of dilated fetal vessels
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• fetal vessels are dilated to a varying degree in placentas; seen by E13.5
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• the fetal vessel parametric length and area is increased relative to the maternal lacunas in the labyrinthine layer at E17
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• homozygotes dying at E17-E18.5 display dilated and congested subpericardial blood vessels
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• at E17.5 or later, homozygotes exhibit enlarged and malformed hearts, with extreme dilation of all chambers and of the thoracic aortae
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• homozygotes dying at E17-E18.5 show hypertrabeculated and thin-walled regions in the right ventricle
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hemorrhage
(
J:20017
)
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• at E19, most live embryos exhibit hemorrhages in subcutaneous regions, periscapular brown fat and the liver
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hematopoietic system
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• at E19, homozygotes display reduced hematocrits relative to wild-type mice
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• at E19, homozygotes display reduced hemoglobin values relative to wild-type mice
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• at E19, homozygotes exhibit a ~20% increase in MCV
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• at E19, homozygotes show a ~85% reduction in thrombocyte counts relative to wild-type mice
• no differences in granulocyte, lymphocyte, and monocyte differential cell counts are observed
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respiratory system
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• all live homozygotes delivered by Cesarian section gasp for air a few times, then cease to breathe and die
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liver/biliary system
small liver
(
J:20017
)
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• at E17.5 or later, homozygotes exhibit reduced liver volume
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homeostasis/metabolism
skin edema
(
J:20017
)
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• homozygotes dying at E17-E18.5 display a generalized subcutaneous edema
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embryo
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• fetal vessels are dilated to a varying degree in placentas; seen by E13.5
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• the fetal vessel parametric length and area is increased relative to the maternal lacunas in the labyrinthine layer at E17
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• nonendothelial labyrinthine cell number, dominated by the trophoblasts, is reduced by about 40%
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nervous system
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• many glia cells that are juxtaposed to the endothelial cells of vessels exhibit prominent swelling
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muscle
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• at E16.5-E19, homozygotes display a dilated aorta with an extended, thinner aortic muscular wall
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integument
skin edema
(
J:20017
)
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• homozygotes dying at E17-E18.5 display a generalized subcutaneous edema
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growth/size/body
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• at E17.5 or later, homozygotes exhibit enlarged and malformed hearts, with extreme dilation of all chambers and of the thoracic aortae
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cellular
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• at E17.5 or later, mutant glomeruli show absence or reduction of mesangial cells in the presence of a morphologically normal basement membrane
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• at E17.5 or later, mutant glomeruli may show absence of mesangial cells
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