It is worthy to note that tubulointerstitial fibrosis is both a key feature of disease progression and a therapeutic target of AS, as a previous report demonstrated that, despite the persistent ultrastructural defect in GBM, a pre-emptive pharmacologic intervention attenuated inflammation and tubulointerstitial fibrosis, and subsequently delayed the onset of end-stage renal disease in Col4a3−/− mice [6]. The gene discussed is COL4A3; the disease is chronic kidney disease.