muscle
• thin myocardium is seen at E18.5, however this abnormality is normalized by 1 month of age
|
cardiovascular system
• E15.5 embryos show irregular capillary diameter with microaneurysms in the brain
• capillaries in the striatum with associated pericytes are straighter and more uniform in diameter
• in the cerebellum, see a reduction of capillary density in the gray matter and the presence of numerous enlarged capillaries
|
• in the striatum, capillary density is lower, but numerous tortuous capillaries with increased diameter are seen in capillary regions not associated with pericytes
• in the cerebellum, see a reduction of capillary density in the gray matter and the presence of numerous enlarged capillaries
|
• E18.5 glomeruli show dilation of the remaining capillary loops
• 3-week old mutants show an increase in the diameter of individual capillary loops
|
• in the striatum, capillary density is lower, but numerous tortuous capillaries with increased diameter are seen in capillary regions not associated with pericytes
|
• microaneurysm formation in the brain
|
• in mutants with greater than 50% of normal overall CNS pericyte density, the retinal vasculature displays irregular microvessel diameter, microaneurysms, and increased vascular regression
• in mutants with less than 50% of normal pericyte density, the retinas develop regions with massive increase of abnormal vessels extending into the vitreous and choroid
|
• exhibit a variable reduction in pericyte density in CNS vessels, affecting arteries, veins and capillaries
(J:78544)
• retinas in mutants with the lowest overall CNS pericyte density however display focal regions of increased pericyte density
(J:78544)
• exhibit a significant reduction in pericyte density in E15.5 embryos that persists into adulthood
(J:89186)
|
• placental defects at E18.5 include dilation of both fetal and maternal vessels due to a reduction in the number of pericytes and trophoblasts
|
• thin myocardium is seen at E18.5, however this abnormality is normalized by 1 month of age
|
• show scattered small hemorrhages deep in the cerebral parenchyma; bleeding seems to start from capillary branching points
|
vision/eye
• mutants with less than 52% of the normal pericyte density in the cerebellum show typical hallmarks of proliferative retinopathy, affecting at least one eye
• retinas of mutants with the lowest overall CNS pericyte density are contracted and often attached to the retinal pigment epithelial cells and the lens
|
• in mutants with greater than 50% of normal overall CNS pericyte density, the retinal vasculature displays irregular microvessel diameter, microaneurysms, and increased vascular regression
• in mutants with less than 50% of normal pericyte density, the retinas develop regions with massive increase of abnormal vessels extending into the vitreous and choroid
|
• regions with high pericyte density show loss of organization of the neural layers and folding of the photoreceptor layer producing typical photoreceptor rosette profiles
|
embryo
• placental defects at E18.5 include dilation of both fetal and maternal vessels due to a reduction in the number of pericytes and trophoblasts
|
homeostasis/metabolism
albuminuria
(
J:89186
)
• mutants older than 12 months develop mild but significant increases in albumin content in urine
|
nervous system
• microaneurysm formation in the brain
|
• show scattered small hemorrhages deep in the cerebral parenchyma; bleeding seems to start from capillary branching points
|
• postnatal brains show increased density of microglial cells at sites of bleeding
|
• postnatal brains show increased density of microglial cells and upregulated expression of glial fibrillary acidic protein at sites of bleeding, hallmarks of reactive gliosis
|
renal/urinary system
albuminuria
(
J:89186
)
• mutants older than 12 months develop mild but significant increases in albumin content in urine
|
• E18.5 glomeruli show a reduction in tuft complexity and dilation of the remaining capillary loops
• 3-week old mutants show glomerular dilation, both an increased glomerulus diameter and an increase in the diameter of the individual capillary loops, however by 6 and 21 months of age, no signs of increased glomerular pathology are seen
|
• E18.5 glomeruli show dilation of the remaining capillary loops
• 3-week old mutants show an increase in the diameter of individual capillary loops
|
• most glomeruli show a reduced mesangial core, although some completely lack mesangial cells at E18.5
• the mesangial deficiency is largely corrected at 3 weeks of age
|
hematopoietic system
• postnatal brains show increased density of microglial cells at sites of bleeding
|
immune system
• postnatal brains show increased density of microglial cells at sites of bleeding
|
cellular
• most glomeruli show a reduced mesangial core, although some completely lack mesangial cells at E18.5
• the mesangial deficiency is largely corrected at 3 weeks of age
|