Phenotypes Associated with This Genotype

Genotype
MGI:6316681

• Allelic Composition: Nphs2^tm2.1Antc/Nphs2^tm3.1Antc; Tg(CAG-cre/Esr1*)86Lbgn/0
• Genetic Background: involves: 129S2/SvPas * C57BL/6 * DBA

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

Nphs2^tm2.1Antc/Nphs2^tm3.1Antc Tg(CAG-cre/Esr1*)86Lbgn/0 mice develop focal-segmental glomerulosclerosis

mortality/aging

premature death ( J:245693 )

• the median survival of tamoxifen-treated mice is 12 weeks

growth/size/body

decreased body weight ( J:245693 )

• mice exhibit lower body weight from week 6 onward after tamoxifen treatment

cardiovascular system

increased systemic arterial blood pressure ( J:245693 )

• blood pressure moderately increases after tamoxifen treatment

homeostasis/metabolism

increased urine creatinine level ( J:245693 )

• mice exhibit increased creatinine levels within a few days of tamoxifen treatment

increased circulating cholesterol level ( J:245693 )

• mice exhibit hypercholesterolemia 4 weeks after tamoxifen induction

decreased circulating serum albumin level ( J:245693 )

• mice exhibit hypoalbuminemia 4 weeks after tamoxifen induction

increased urine protein level ( J:245693 )

• mice develop proteinuria within a few days of tamoxifen treatment which increases to a maximum 4-5 weeks after induction and thereafter decreases gradually but remains elevated compared to controls

renal/urinary system

increased urine creatinine level ( J:245693 )

• mice exhibit increased creatinine levels within a few days of tamoxifen treatment

increased urine protein level ( J:245693 )

• mice develop proteinuria within a few days of tamoxifen treatment which increases to a maximum 4-5 weeks after induction and thereafter decreases gradually but remains elevated compared to controls

abnormal podocyte foot process morphology ( J:245693 )

• foot processes are irregularly shaped one week after tamoxifen induction, progressing to effacement

fused podocyte foot processes ( J:245693 )

• foot processes are fused one week after tamoxifen induction progressing to global fusion and effacement

podocyte foot process effacement ( J:245693 )

• in tamoxifen treated mice

decreased podocyte number ( J:245693 )

• mice show reduced podocyte number per glomerulus starting at the end of the second week after tamoxifen induction (68% of controls) to 18% of controls at 12-16 weeks

increased renal glomerulus basement membrane thickness ( J:245693 )

• glomerular basement membrane is thickened in tamoxifen-induced mice

glomerulosclerosis ( J:245693 )

• mice exhibit early glomerular sclerosis form the first week after tamoxifen induction that progresses increases until week eight when 100% of glomeruli are partially or globally sclerosed

renal interstitial fibrosis ( J:245693 )

• mice develop severe interstitial fibrosis which is visible from the first week after tamoxifen induction and increase to 12% of the total kidney area in the course of the disease

renal tubule atrophy ( J:245693 )

• mice develop tubular atrophy that is visible from the first week after tamoxifen induction

decreased creatinine clearance ( J:245693 )

• mice exhibit diminished creatinine clearance 4 weeks after tamoxifen induction

kidney failure ( J:245693 )

• mice progress to renal failure after tamoxifen-induction

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
nephrotic syndrome		DOID:1184		J:245693