FBLN1 and autosomal dominant polycystic kidney disease: Similarly, ECM-remodelling enzymes TIMP1, TIMP2, TIMP3, MMP7, ADAM17, and matricellular proteins CCN1, CCN2, SPARC, FBLN1 and Serpine-1 were predominantly expressed in ADPKD myofibroblasts (Fig 2C, D).