Mouse models of autosomal recessive AS with mutations of Col4a3 or both Col4a3 and Col4a4 genes exhibit glomerular basement membrane (GBM) lesions that mimic the human disease and present with delayed onset glomerulonephritis that progresses to end-stage renal failure (ESRF) and early death. The gene discussed is COL4A3; the disease is stage 5 chronic kidney disease.