All Phenotypes Cd151<tm1Nki> MGI Mouse

weight loss ( J:114988 )

• exhibit substantial loss of body weight before 9 months of age

increased urine protein level ( J:114988 )

• develop proteinuria before 3 weeks of age which progresses to massive proteinuria with age

• the onset and degree of proteinuria are variable

albuminuria ( J:114988 )

• urinary albumin increases with age and reaches a plateau at 6 wks of age

tubulointerstitial nephritis ( J:114988 )

• focal interstitial inflammation in close proximity to interlobular blood vessels

abnormal kidney morphology ( 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

abnormal renal glomerular capsule morphology ( J:114988 )

• capsules are granulated because of cortical degeneration in severely affected kidneys

absent podocyte foot process ( J:114988 )

• exhibit loss of podocyte foot processes

podocyte foot process effacement ( J:114988 )

• foot processes in contact with the abnormal GBM are effaced

absent podocyte slit diaphragm ( J:114988 )

• exhibit complete loss of glomerular epithelial slit diaphragm architecture

• glomerulosclerosis precedes the loss of glomerular epithelial slit diaphragm components

abnormal renal glomerulus basement membrane morphology ( J:114988 )

• glomerular basement membrane (GBM) of some capillary loops is disorganized, laminated, abnormally thick and forms extensive spikes

increased renal glomerulus basement membrane thickness ( J:114988 )

• GBM of some capillary loops is abnormally thick

abnormal renal glomerulus morphology ( J:114988 )

abnormal glomerular capillary morphology ( J:114988 )

• capillary collapse in severely affected kidneys

abnormal glomerular capillary endothelium morphology ( J:114988 )

• the capillary endothelium is swollen

abnormal glomerular endothelium fenestra morphology ( J:114988 )

• fenestrations are occasionally lost

increased mesangial cell number ( J:114988 )

• mesangial hypercellularity

expanded mesangial matrix ( J:114988 )

• show expansion of the mesangial matrix

glomerulosclerosis ( J:114988 )

• exhibit focal glomerulosclerosis; glomeruli are segmentally or globally sclerosed with extensive deposits of basement membrane components

glomerular crescent ( J:114988 )

• parietal epithelial crescents in severely affected kidneys

renal glomerular synechia ( J:114988 )

• tuft adhesions to Bowman's capsule with extracapillary cell proliferation and fibrosis are observed

renal interstitial fibrosis ( J:114988 )

• exhibit interstitial and periglomerular fibrosis

kidney atrophy ( J:114988 )

• severely affected kidneys are contracted

abnormal proximal convoluted tubule morphology ( J:114988 )

• proximal tubules either show degeneration of varying severity or are dilated

dilated proximal convoluted tubule ( J:114988 )

• proximal tubules are either dilated or show degeneration of varying severity

renal cast ( J:114988 )

• in severe cases, proximal tubules exhibit protein casts indicative of proteinuria

granular kidney ( J:114988 )

• severely affected kidneys have a granular surface while mildly affected kidneys show a relatively smooth and intact surface

kidney degeneration ( J:114988 )