Collagen is a pivotal ECM component [65], and procollagen 1Aα- and 3Aα-dependent cellular processes are implicated in IPF [66], together with an aberrant CTGF increase, a key step in the formation of new ECM, and the assembly of a fibrillar fibronectin matrix, which serves as a scaffold for the binding of collagens and other ECM proteins [67]. Here, CCN2 is linked to idiopathic pulmonary fibrosis.