Phenotypes for Tg(Nphs1-cre)33Mska Tjp1 MGI:5770415 MGI Mouse

postnatal growth retardation ( J:226210 )

• significant growth retardation at 6 weeks of age

detached podocyte ( J:226210 )

• impaired adhesion of the podocyte foot processes to the GBM at 2 weeks but not at 1 week of age

increased urine protein level ( J:226210 )

• significant proteinuria at 2 weeks of age

• massive proteinuria at 6 weeks of age

• albumin and higher molecular weight proteins detected in urine at P0

albuminuria ( J:226210 )

• albumin is detected in the urine at P0

abnormal kidney morphology ( J:226210 )

• disordered tissue architecture at 6 weeks of age, as shown by PAS staining

abnormal podocyte foot process morphology ( J:226210 )

• severe disorganization and destruction of the foot processes at 6 weeks of age

• disorganized interdigitation at 1 week of age

• loss of interdigitation at 2 weeks of age

• foot processes are located close together in maturing podocytes at P0

fused podocyte foot processes ( J:226210 )

• aberrant contacts between the foot processes at 2 weeks of age

podocyte foot process effacement ( J:226210 )

• global foot process effacement at 2 weeks of age

abnormal podocyte slit diaphragm morphology ( J:226210 )

• abnormal distribution of the slit diaphragm components at 1 week and 2 weeks of age

• absence of normal slit diaphragms in maturing podocytes in P0

absent podocyte slit diaphragm ( J:226210 )

• loss of the slit diaphragm at 2 weeks of age

increased renal glomerulus basement membrane thickness ( J:226210 )

• abnormally thickened, tortuous GBM at 6 weeks of age

• however, GBM organization is largely normal at 2 weeks of age

abnormal glomerular filtration barrier morphology ( J:226210 )

• impaired formation and maintenance of the podocyte filtration barrier

glomerulosclerosis ( J:226210 )

• severe glomerulosclerosis at 4 weeks of age, with milder but obvious glomerular defects at 2 weeks of age

• global sclerosis (>90%) with extensive deposits of basement membrane components, as shown by Jones silver and Massons trichrome staining, at 6 weeks of age

abnormal kidney pelvis morphology ( J:226210 )

• enlarged renal pelvis at 6 weeks but not at 2 weeks of age

kidney papillary atrophy ( J:226210 )

• atrophic renal papilla at 6 weeks but not at 2 weeks of age

dilated renal tubule ( J:226210 )

• dilated renal tubules at 4 and 6 weeks of age

renal cast ( J:226210 )

• proteinaceous casts within dilated renal tubules at 4 weeks of age

granular kidney ( J:226210 )

• kidneys have a more granular surface at 6 weeks of age

pale kidney ( J:226210 )

• pale kidneys at 6 weeks of age