The main finding of this study was that the specific iBET JQ1 reduced glomerular COLIV accumulation in experimental GN, as shown in murine NTS–induced glomerular damage through the following different mechanisms: (1) inhibiting the binding of BET proteins to acetylated residues in the promoter regions of Col4a3, directly inhibiting Col4a3 gene overexpression, and (2) blocking the activation of the transcription factors SOX9 and SMAD3, which are both involved in the regulation of ECM proteins. Here, COL4A3 is linked to ganglioneuroma.