COL4A3 and chronic kidney disease: The use of Col4a3-deficient (Col4a3−/−) mice has been an experimental model of human AS, which causes the development of proteinuria as early as the age of postnatal 6 weeks, with progressive glomerulonephritis and tubulointerstitial scarring, and leads to death with end-stage renal disease at the age of approximately 14 weeks [5].