About   Help   FAQ
Phenotypes Associated with This Genotype
Genotype
MGI:3691144
Allelic
Composition
Cd151tm1Nki/Cd151tm1Nki
Genetic
Background
involves: 129P2/OlaHsd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cd151tm1Nki mutation (0 available); any Cd151 mutation (19 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Glomerular basement membrane abnormalities and foot process effacement in kidneys of mildly affected Cd151tm1Nki/Cd151tm1Nki and Itga3tm1Son/Itga3tm1Son Tg(NPHS2-cre)295Lbh/0 mice

growth/size/body
• exhibit substantial loss of body weight before 9 months of age (J:114988)
• exhibit substantial loss of body weight before 9 months of age (J:114988)

renal/urinary system
• develop proteinuria before 3 weeks of age which progresses to massive proteinuria with age (J:114988)
• the onset and degree of proteinuria are variable (J:114988)
• develop proteinuria before 3 weeks of age which progresses to massive proteinuria with age (J:114988)
• the onset and degree of proteinuria are variable (J:114988)
• urinary albumin increases with age and reaches a plateau at 6 wks of age (J:114988)
• urinary albumin increases with age and reaches a plateau at 6 wks of age (J:114988)
• focal interstitial inflammation in close proximity to interlobular blood vessels (J:114988)
• focal interstitial inflammation in close proximity to interlobular blood vessels (J:114988)
• mice exhbit renal abnormalities that are seen in patients with nephropathy with pretibial epidermolysis bullosa and deafness, however do no develop the typical skin or hearing abnormalities (J:114988)
• mice exhbit renal abnormalities that are seen in patients with nephropathy with pretibial epidermolysis bullosa and deafness, however do no develop the typical skin or hearing abnormalities (J:114988)
• capsules are granulated because of cortical degeneration in severely affected kidneys (J:114988)
• capsules are granulated because of cortical degeneration in severely affected kidneys (J:114988)
• exhibit loss of podocyte foot processes (J:114988)
• exhibit loss of podocyte foot processes (J:114988)
• foot processes in contact with the abnormal GBM are effaced (J:114988)
• foot processes in contact with the abnormal GBM are effaced (J:114988)
• exhibit complete loss of glomerular epithelial slit diaphragm architecture (J:114988)
• glomerulosclerosis precedes the loss of glomerular epithelial slit diaphragm components (J:114988)
• exhibit complete loss of glomerular epithelial slit diaphragm architecture (J:114988)
• glomerulosclerosis precedes the loss of glomerular epithelial slit diaphragm components (J:114988)
• glomerular basement membrane (GBM) of some capillary loops is disorganized, laminated, abnormally thick and forms extensive spikes (J:114988)
• glomerular basement membrane (GBM) of some capillary loops is disorganized, laminated, abnormally thick and forms extensive spikes (J:114988)
• GBM of some capillary loops is abnormally thick (J:114988)
• GBM of some capillary loops is abnormally thick (J:114988)
• capillary collapse in severely affected kidneys (J:114988)
• capillary collapse in severely affected kidneys (J:114988)
• the capillary endothelium is swollen (J:114988)
• the capillary endothelium is swollen (J:114988)
• fenestrations are occasionally lost (J:114988)
• fenestrations are occasionally lost (J:114988)
• mesangial hypercellularity (J:114988)
• mesangial hypercellularity (J:114988)
• show expansion of the mesangial matrix (J:114988)
• show expansion of the mesangial matrix (J:114988)
• exhibit focal glomerulosclerosis; glomeruli are segmentally or globally sclerosed with extensive deposits of basement membrane components (J:114988)
• exhibit focal glomerulosclerosis; glomeruli are segmentally or globally sclerosed with extensive deposits of basement membrane components (J:114988)
• parietal epithelial crescents in severely affected kidneys (J:114988)
• parietal epithelial crescents in severely affected kidneys (J:114988)
• tuft adhesions to Bowman's capsule with extracapillary cell proliferation and fibrosis are observed (J:114988)
• tuft adhesions to Bowman's capsule with extracapillary cell proliferation and fibrosis are observed (J:114988)
• exhibit interstitial and periglomerular fibrosis (J:114988)
• exhibit interstitial and periglomerular fibrosis (J:114988)
• severely affected kidneys are contracted (J:114988)
• severely affected kidneys are contracted (J:114988)
• proximal tubules either show degeneration of varying severity or are dilated (J:114988)
• proximal tubules either show degeneration of varying severity or are dilated (J:114988)
• proximal tubules are either dilated or show degeneration of varying severity (J:114988)
• proximal tubules are either dilated or show degeneration of varying severity (J:114988)
• in severe cases, proximal tubules exhibit protein casts indicative of proteinuria (J:114988)
• in severe cases, proximal tubules exhibit protein casts indicative of proteinuria (J:114988)
• severely affected kidneys have a granular surface while mildly affected kidneys show a relatively smooth and intact surface (J:114988)
• severely affected kidneys have a granular surface while mildly affected kidneys show a relatively smooth and intact surface (J:114988)
• capsules are granulated because of cortical degeneration in severely affected kidneys (J:114988)
• proximal tubules may show degeneration of varying severity (J:114988)
• capsules are granulated because of cortical degeneration in severely affected kidneys (J:114988)
• proximal tubules may show degeneration of varying severity (J:114988)
• develop severe renal failure, caused by progressive abnormalities of the glomerular basement membrane, loss of podocyte foot processes, glomeruloscelrosis, and cystic tubular dilation (J:114988)
• develop severe renal failure, caused by progressive abnormalities of the glomerular basement membrane, loss of podocyte foot processes, glomeruloscelrosis, and cystic tubular dilation (J:114988)

homeostasis/metabolism
• develop proteinuria before 3 weeks of age which progresses to massive proteinuria with age (J:114988)
• the onset and degree of proteinuria are variable (J:114988)
• develop proteinuria before 3 weeks of age which progresses to massive proteinuria with age (J:114988)
• the onset and degree of proteinuria are variable (J:114988)
• urinary albumin increases with age and reaches a plateau at 6 wks of age (J:114988)
• urinary albumin increases with age and reaches a plateau at 6 wks of age (J:114988)

immune system
• focal interstitial inflammation in close proximity to interlobular blood vessels (J:114988)
• focal interstitial inflammation in close proximity to interlobular blood vessels (J:114988)

cardiovascular system
• capillary collapse in severely affected kidneys (J:114988)
• capillary collapse in severely affected kidneys (J:114988)
• the capillary endothelium is swollen (J:114988)
• the capillary endothelium is swollen (J:114988)
• fenestrations are occasionally lost (J:114988)
• fenestrations are occasionally lost (J:114988)

hearing/vestibular/ear
N
• do not exhibit a hearing impairment (J:114988)
• do not exhibit a hearing impairment (J:114988)

integument
N
• skin integrity is not impaired (J:114988)
• skin integrity is not impaired (J:114988)


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
02/02/2016
MGI 6.02
The Jackson Laboratory