Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rhpn1tm1Ktry mutation
(0 available);
any
Rhpn1 mutation
(34 available)
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homeostasis/metabolism
renal/urinary system
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• albuminuria is detected in urine by 2 weeks of age but not in newborns
• Background Sensitivity: mice on the congenic C57BL/6 background are less albuminuric than mice on the mixed background
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rhpn1tm1Ktry mutation
(0 available);
any
Rhpn1 mutation
(34 available)
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homeostasis/metabolism
renal/urinary system
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• increase in kidney size due to increase in glomerular size
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• heterogeneity in the progression of albuminuria is seen on the mixed background, with mice presenting albuminuria by 2 weeks of age and the majority of mice having severe albuminuria by 2-4 months of age
• Background Sensitivity: mice on the mixed background are more albumuminuric than mice on the congenic C57BL/6 background
• however, serum creatinine levels are unchanged and no increased mortality is seen
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• primary podocytes show ventral stress fibers that span across the cell body and do not adopt the same convex morphology and concentrated peripheral actin network seen in wild-type podocytes
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• at the basal aspect of podocyte processes abutting the glomerular basement membrane, membrane invaginations are irregularities can be seen
• severely albuminuric mice show a loss of the slender foot processes and their replacement with broader, stubby processes that have microvillus transformation on the apical surface
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• reduction in the number of slits circumnavigating capillary loops
• slit diaphragms are occasionally seen spanning the gap between two adjacent foot processes
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• severely albuminuric mice show a loss of the slender foot processes and their replacement with broader, stubby processes that have microvillus transformation on the apical surface
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• thickening and remodeling of the glomerular basement membrane that accompanies food process effacement
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• glomerular damage, both focal and segmental
• increase in glomerular size is seen before glomeruli become atrophic and sclerotic
• glomeruli from 1-week old mice tend to have areas of food process effacement and a reduction in slit frequency that are more prominent than glomerular basement membrane changes
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• early on, mesangial expansion is seen, with no changes in the tubular compartment of kidneys
• mice with severe albuminuria at 2 months show increased mesangial expansion
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• mice with severe albuminuria at 2 months of age show prominent sclerotic lesions consistent with focal segmental glomerulosclerosis
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• mice with severe albuminuria at 2 months show renal glomerular synechia
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• protein casts are seen in tubular lumens of mice with macroalbuminuria
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growth/size/body
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• increase in kidney size due to increase in glomerular size
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rhoatm1Jrel mutation
(0 available);
any
Rhoa mutation
(70 available)
Rhpn1tm1Ktry mutation
(0 available);
any
Rhpn1 mutation
(34 available)
Tg(NPHS2-cre)295Lbh mutation
(3 available)
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growth/size/body
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• reduced body weight by 3 months of age
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homeostasis/metabolism
renal/urinary system
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• mice exhibit a more severe kidney injury than single Rhpn1 homozygotes
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• complete podocyte effacement
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• segmental and global focal segmental glomerulosclerosis
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rhpn1tm1Ktry mutation
(0 available);
any
Rhpn1 mutation
(34 available)
Rhpn2tm1Ssch mutation
(0 available);
any
Rhpn2 mutation
(40 available)
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mortality/aging
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• mice show reduced lifespan
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homeostasis/metabolism
renal/urinary system
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• albumin/creatinine ratio is increased
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• segmental and global glomerular sclerosis
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• interstitial fibrosis in nephrotic kidneys
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• tubule dilatation in nephrotic kidneys
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• tubular casts in nephrotic kidneys
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