Phenotypes Associated with This Genotype

Genotype
MGI:4847559

• Allelic Composition: Nphs2^tm1Antc/Nphs2^tm3.1Antc; Tg(CAG-cre/Esr1*)86Lbgn/0
• Genetic Background: involves: 129 * C57BL/6 * DBA

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

mortality/aging

premature death ( J:166320 )

• mice die 11 weeks after tamoxifen treatment

renal/urinary system

increased urine protein level ( J:166320 )

• after 4 weeks of tamoxifen treatment, mice develop nonselective proteinuria compared with control mice

albuminuria ( J:166320 )

• after 10 days of tamoxifen treatment

• after 2 weeks of nursing from mice fed tamoxifen

abnormal kidney morphology ( J:166320 )

• after 9 weeks, tamoxifen-treated mice exhibit progressive tubular injury with basement membrane thickening and interstitial fibrosis unlike similarly treated wild-type mice

podocyte foot process effacement ( J:166320 )

• tamoxifen-treated mice exhibit focal effacement at 1 and 2 weeks then diffuse effacement at 4 weeks unlike similarly treated wild-type mice

podocyte hypertrophy ( J:166320 )

• after 2 weeks of tamoxifen treatment, mice exhibit podocyte hypertrophy compared with similarly treated control mice

abnormal renal glomerulus morphology ( J:166320 )

• after 4 weeks, tamoxifen-treated mice exhibit glomerular pseudocrescents in some glomeruli unlike in similarly treated control mice

increased mesangial cell number ( J:166320 )

• mice nursed by dams fed tamoxifen exhibit lesions of mesangial proliferation compared with similarly treated controls

expanded mesangial matrix ( J:166320 )

• after 2 weeks of tamoxifen treatment, mice exhibit minimal mesangial matrix expansion compared with similarly treated control mice

glomerulosclerosis ( J:166320 )

• after 4 weeks of tamoxifen treatment, mice exhibit focal segmental glomerulosclerosis in many glomeruli with varying severity unlike in similarly treated control mice

• mice nursed by dams fed tamoxifen exhibit lesions of focal segmental glomerulosclerosis unlike similarly treated control mice

• glomerulosclerosis worsens by 6 weeks of tamoxifen treatment

• at or near death, tamoxifen-treated mice exhibit global scelrosis

renal interstitial fibrosis ( J:166320 )

• after 9 weeks in tamoxifen-treated mice

abnormal renal tubule morphology ( J:166320 )

• tamoxifen-treated mice exhibit tubulointerstitial injury with diffuse tubular dilation, tubular atrophy and necrosis, and proteinaceous casts unlike in similarly treated control mice

renal tubular necrosis ( J:166320 )

• in tamoxifen-treated mice

renal tubule atrophy ( J:166320 )

• in tamoxifen-treated mice

dilated renal tubule ( J:166320 )

• in tamoxifen-treated mice

renal cast ( J:166320 )

• in tamoxifen-treated mice

homeostasis/metabolism

increased circulating creatinine level ( J:166320 )

• after 6 weeks of tamoxifen treatment

increased blood urea nitrogen level ( J:166320 )

• after 6 weeks of tamoxifen treatment

increased circulating cholesterol level ( J:166320 )

• after 4 weeks of tamoxifen treatment

increased urine protein level ( J:166320 )

• after 4 weeks of tamoxifen treatment, mice develop nonselective proteinuria compared with control mice

albuminuria ( J:166320 )

• after 10 days of tamoxifen treatment

• after 2 weeks of nursing from mice fed tamoxifen

cardiovascular system

increased systemic arterial systolic blood pressure ( J:166320 )

• modestly after 4 weeks of tamoxifen treatment

cellular

increased mesangial cell number ( J:166320 )

• mice nursed by dams fed tamoxifen exhibit lesions of mesangial proliferation compared with similarly treated controls

renal tubular necrosis ( J:166320 )

• in tamoxifen-treated mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
nephrotic syndrome		DOID:1184		J:166320