Analysis Tools
homeostasis/metabolism
albuminuria
(
J:84701
)
|
• apparent in most at 3 months of age
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renal/urinary system
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• formation of ballooning glomerular capillaries in late gestation
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albuminuria
(
J:84701
)
|
• apparent in most at 3 months of age
|
|
• delayed formation of the renal glomerulus mesangium
|
|
• embryos exhibit a reduction in the number of mesangial cells in glomeruli at late gestation, leading to the formation of ballooning glomerular capillaries
• the mesangial cell deficiency is temporary and by 1 month of age, most glomeruli are normalized
|
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• by 6 months of age, mice show extensive accumulation of extracellular matrix
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vision/eye
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• about 10% display white ocular opacities
|
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• 100% penetrance
|
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• vascular remodeling occurs but the pattern of remodeled vessels is abnormal, with a shortage of branch points
• the retinal vasculature is severely disorganized and retinal vessels are abnormal from the onset of sprouting
• plexus formation is delayed and asymmetric at P2, the vascular plexuses at P5 are highly irregular and display fewer sprouts and sites with increased as well as decreased vascular density
• retinal plexuses show a significant reduction in the pericyte density and pericytes in the retina are partially detached from the endothelium
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• invasion of retinal pigment epithelial cells
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• degeneration of the nuclear layer
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• degeneration of the photoreceptor layer
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• fibrosis and traction
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cardiovascular system
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• abnormal capillary morphology in postnatal mice
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• formation of ballooning glomerular capillaries in late gestation
|
|
• the retinal vasculature is severely disorganized and retinal vessels are abnormal from the onset of sprouting
• plexus formation is delayed and asymmetric at P2, the vascular plexuses at P5 are highly irregular and display fewer sprouts and sites with increased as well as decreased vascular density
• vascular remodeling occurs but the pattern of remodeled vessels is abnormal, with a shortage of branch points
• retinal plexuses show a significant reduction in the pericyte density and pericytes in the retina are partially detached from the endothelium
|
|
• at E15.5, the number of pericytes in the forebrain is reduced to about 50% of normal
• pericytes in the brain are partially dissociated from the abluminal endothelial surface and protrude cellular processes away from the vessel
• pericytes in the retina are partially detached from the endothelium
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• vessel-associated vascular smooth muscle cells are present but abnormally organized in the retina
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growth/size/body
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• slightly growth retarded
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nervous system
pigmentation
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• invasion of retinal pigment epithelial cells
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reproductive system
muscle
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• vessel-associated vascular smooth muscle cells are present but abnormally organized in the retina
|
cellular
|
• embryos exhibit a reduction in the number of mesangial cells in glomeruli at late gestation, leading to the formation of ballooning glomerular capillaries
• the mesangial cell deficiency is temporary and by 1 month of age, most glomeruli are normalized
|
