Tissue inhibitor of metalloproteinase 1 is highly induced during fibrosis in a number of animal models, such as bleomycin- and paraquat-induced lung fibrosis, and in human fibrotic diseases, such as IPF and liver cirrhosis, implicating TIMP1 and its upregulated expression in fibrosis development (38, 98–101). This evidence concerns the gene TIMP1 and idiopathic pulmonary fibrosis.