Similar with the patient, Del-ATGG male mice showed hematuria, loss of expression of Col4a5 in renal tissues, typical AS-like basement membrane alternation, and other histopathological findings of increased glomerular volume, mesangial hyperplasia, focal glomerular sclerosis, tubular atrophy, and interstitial fibrosis. Here, COL4A5 is linked to focal segmental glomerulosclerosis.