Another target of miR-101 with profibrotic effects is endothelin-1; this protein is elevated in the serum of patients with IPF and has many profibrotic effects, like inducing the production of collagen I and III, as well as the overproduction of CTGF, fibronectin, α-smooth muscle actin [167,168,169,170]. The gene discussed is CCN2; the disease is idiopathic interstitial pneumonia.