Figure 7. Col4 α−/ − hereditary nephritis model.
Mouse harboring a genetic mutation in the gene that encodes the glomerular basement membrane protein collagen IV alpha 3 (col4α3−/ −) develop hereditary nephritis with proteinuria and progressive renal failure. The tempo of the kidney disease is mouse strain-dependent. Compared to the asymptomatic heterozygous littermates (col4α3+/−) the mutant mice develop progressive interstitial fibrosis (shown in the Masson trichrome-stained photomicrographs), reduced tubular volume and interstitial volume expansion by matrix proteins. Data are from a study in 10-week old 129Sv/J mice that also harbor an asymptomatic heterozygous mutation in the 6 integrin gene that are reproduced from [27] with copyright permission.