renal/urinary system
| N |
• at 14 months of age, no significant differences in GFR, renal blood flow, renal vascular resistance, filtration fraction, fractional sodium and potassium excretions, urinary protein concentration, and kidney weight or volume are observed
(J:103081)
• at 26 weeks of age, heterozygotes show normal renal innervation relative to wild-type controls
(J:168650)
• at 26 weeks, lower nephron number is not accompanied by changes in intrarenal arteries or peritubular capillaries or by altered distribution of tubular transporters
(J:168650)
|
|
• at 26 weeks of age, glomerular cell proliferation is significantly higher than that in wild-type controls
|
|
• at 14 months of age, one kidney displayed a cortical cyst
|
|
• at 14 months of age, one kidney displayed areas of focal interstitial inflammation
|
|
• at 14 months of age, mean renal corpuscle volume is 30% larger than in wild-type kidneys; however, total glomerular and renal corpuscle volumes remain unaffected
|
|
• at 26 weeks of age, the number of endothelial cells per glomerulus is significantly increased relative to that in wild-type controls; however, endothelial cells are not hypertrophic
|
|
• at 26 weeks of age, glomerular capillary density i.e. capillary length per glomerular volume is significantly reduced (-14%) relative to that in wild-type controls; however, the total length of glomerular capillaries per kidney is normal
|
|
• at 26 weeks of age, the number of mesangial cells per glomerulus is significantly increased relative to that in wild-type controls; however, mesangial cells are not hypertrophic
|
|
• at 26 weeks of age, enlargement of podocytes with increased cytoplasmic vacuolization is observed
• a slightly higher desmin positivity suggests mild podocyte damage
|
|
• at 26 weeks of age, partial fusion of foot processes is observed
|
|
• at 26 weeks of age, the podocyte number per area is significantly lower (-30%) than that in wild-type controls, indicating podocyte rarefaction
|
|
• at 26 weeks of age, significant thickening of the GBM is observed relative to wild-type controls
|
|
• at 14 months of age, heterozygotes contain ~30% fewer glomeruli than wild-type mice
|
|
• at 14 months of age (but not at P30), glomeruli of heterozygous kidneys are 20% larger than those of wild-type kidneys; however, no evidence of sclerosis or hypercellularity is observed
(J:103081)
• at 26 weeks of age, mean glomerular volume is significantly higher (+49.5%) than in wild-type controls; however, total glomerular volume is comparable between the groups
(J:168650)
|
|
• at 26 weeks of age, the % of interstitial fibrous tissue is significantly higher than in wild-type controls, indicating early interstitial activation
• however, no proinflammatory or prohypertensive changes are observed in the kidney
|
|
• at 26 weeks of age, absolute and relative kidney weights are significantly lower than in wild-type controls
|
|
• in heterozygotes with a single kidney, a compensatory higher volume of the single kidney is seen at 26 weeks, so that the relative total kidney weight is similar to that in mice with two kidneys
|
|
• at 26 weeks of age, nephron number is reduced by 32.8% relative to that in wild-type controls
(J:168650)
|
|
|
|
• at 14 months of age, male heterozygotes exhibit ~30% fewer nephrons than wild-type mice
(J:103081)
|
|
• at 14 months of age, heterozygotes display greater and more widespread renal tubular vacuolation than wild-type mice; however, no signs of tubular necrosis or apoptosis are observed
|
|
• at 26 weeks of age, 5 of 11 female and 2 of 11 male heterozygotes exhibit unilateral renal agenesis
|
cardiovascular system
| N |
• at 26 weeks of age, heterozygotes show no significant differences in mean arterial pressure or relative heart weight compared to wild-type controls
|
|
• at 26 weeks of age, the number of endothelial cells per glomerulus is significantly increased relative to that in wild-type controls; however, endothelial cells are not hypertrophic
|
|
• at 26 weeks of age, glomerular capillary density i.e. capillary length per glomerular volume is significantly reduced (-14%) relative to that in wild-type controls; however, the total length of glomerular capillaries per kidney is normal
|
|
• at 14 months of age, anesthetized heterozygotes display a 18 mm Hg increase in mean arterial pressure relative to wild-type littermates
|
immune system
|
• at 14 months of age, one kidney displayed areas of focal interstitial inflammation
|
cellular
|
• at 26 weeks of age, the number of mesangial cells per glomerulus is significantly increased relative to that in wild-type controls; however, mesangial cells are not hypertrophic
|
|
• at 26 weeks of age, glomerular cell proliferation is significantly higher than that in wild-type controls
|
growth/size/body
|
• at 14 months of age, one kidney displayed a cortical cyst
|


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