Mouse Genome Informatics
hm
    Col4a3tm1Dec/Col4a3tm1Dec
129-Col4a3tm1Dec/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Sostdc1tm1Myan/Sostdc1tm1Myan Col4a3tm1Dec/Col4a3tm1Dec mice show less glomerular and tubular injury than Col4a3tm1Dec/Col4a3tm1Dec mice

mortality/aging
• impaired survival compared to Col4a3tm1Dec Sostdc1tm1Myan double homozygotes beyond 13 weeks of age
• impaired survival compared to Col4a3tm1Dec Sostdc1tm1Myan double homozygotes beyond 13 weeks of age

renal/urinary system
• intraglomerular hemorrhage at 6 weeks of age
• at 5 weeks of age, proteinuria is initiated
• at 10 weeks of age, severe glomerular lesions associated with tubulointerstitial fibrosis are observed
• irregular thickening and splitting of the glomerular basement membrane at 4 weeks of age by electron microscopy
• irregular thickening at 4 weeks of age
• at 6 weeks of age, minor glomerular lesions are occasionally observed by light microscopy
• progressive glomerulonephritis
• segmental sclerosis at 6 weeks of age
• at 10 weeks of age mice demonstrate glomerulosclerosis associated with inflammatory cell infiltration, interstitial fibrosis, tubular atrophy, and cast formation
• at 10 weeks of age mice demonstrate tubular atrophy
• renal cast formation at 10 weeks of age
• at 10 weeks of age renal function is deteriorating

homeostasis/metabolism
• at 5 weeks of age, proteinuria is initiated
• at 10 weeks of age, severe glomerular lesions associated with tubulointerstitial fibrosis are observed

immune system
• progressive glomerulonephritis

cardiovascular system
• intraglomerular hemorrhage at 6 weeks of age

Mouse Models of Human Disease
OMIM IDRef(s)
Alport Syndrome, Autosomal Recessive 203780 J:158731