Interestingly, subsequent studies identified that both the bleomycin-induced mice pulmonary fibrosis and lung specimens from patients with IPF upregulated the known Notch-related genes [26, 38] and mesenchymal-specific conditional Notch1-deficient mice reduced bleomycin-induced pulmonary fibrosis [39]. Here, NOTCH1 is linked to idiopathic pulmonary fibrosis.