Phenotypes for Prkci Tg(Nphs1-cre)33Mska MGI:5295616 MGI Mouse

premature death ( J:144995 )

• mice exhibit a reduced lifespan with a median age of death of 6 weeks

postnatal growth retardation ( J:144995 )

• mice exhibit growth retardation by 4 weeks of age

detached podocyte ( J:144995 )

• partial detachment of podocytes with an irregular pattern of nephrin staining is seen at P10

increased urine protein level ( J:144995 )

• mice exhibit massive proteinuria at P0 and throughout their lives

• however, kidneys appear grossly normal at P0

abnormal podocyte morphology ( J:144995 )

fused podocyte foot processes ( J:144995 )

• at P10, irregular adhesions between foot processes are observed

podocyte foot process effacement ( J:144995 )

• at P10, effacement of foot processes is observed

• however, fine foot processes are noted at P0

abnormal podocyte polarity ( J:144995 )

• at P7-P10, the apico-basal cell polarity of podocytes is disturbed

• at P7, podocalyxin loses its apically restricted localization, whereas ZO-1 localizes at the irregular cell-cell junctions of podocytes

abnormal podocyte slit junction morphology ( J:144995 )

• at P10, irregular adhesions between foot processes do not form tight junctions

abnormal podocyte slit diaphragm morphology ( J:144995 )

• at P7-P10, apically dislocated slit diaphragms are observed

• however, normal slit diaphragms are noted at P0

decreased podocyte number ( J:144995 )

• loss of podocytes is noted by P21

abnormal renal glomerulus morphology ( J:144995 )

• at P10, glomeruli display partial detachment of podocytes, occasional adhesion of glomeruli to Bowman's capsules, mesangial expansion, dilated capillaries, and an irregular pattern of nephrin staining

dilated glomerular capillary ( J:144995 )

• at P10, glomerular capillaries are dilated

expanded mesangial matrix ( J:144995 )

• at P10

glomerulosclerosis ( J:144995 )

• by P21, glomeruli exhibit advanced segmental to global sclerotic lesions, consistent with severe renal dysfunction

renal glomerular synechia ( J:144995 )

• at P10, occasional adhesion of glomeruli to Bowman's capsules is observed

abnormal proximal convoluted tubule morphology ( J:144995 )

• at P0, PAS staining indicates occasional hyaline droplets, representing reabsorbed urinary protein, in proximal renal tubules

renal cast ( J:144995 )

• at P0, PAS staining indicates occasional hyaline droplets, representing reabsorbed urinary protein, in proximal renal tubules

increased circulating creatinine level ( J:144995 )

• mice display a significantly increased serum creatinine level by 3 weeks of age

increased blood urea nitrogen level ( J:144995 )

• mice display a significantly increased blood urea nitrogen level by 3 weeks of age