Pirfenidone
Antifibrotic / Antifibrotic agent. Prevents lung fibrosis in bleomycin-induced animal models.1 Down-regulates bleomycin-induced overexpression of lung procollagen I and III genes.2 Up-regulates RGS2 (Regulator of G-protein Signaling 2) which represents a new mechanism of pirfenidone action.3 Inhibits fibroblast proliferation.4 Suppresses TNFα production at the translational level.5 Scavenges hydroxyl radicals and inhibits lipid peroxidation in a dose-dependent manner.6 Recently approved therapeutic agent for idiopathic pulmonary fibrosis.7 Orally active.
Biochemicals & reagents
53179-13-8
1) Kehrer and Margolin (1997), Pirfenidone diminishes cyclophosphamide-induced lung fibrosis in mice; Toxicol.Lett., 90 125 2) Iyet et al. (1999), Effects of pirfenidone on procollagen gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis; J.Pharmacol.Exp.Ther., 289 211 3) Xie et al. (2002), Upregulation of RGS2: a new mechanism for pirfenidone amelioration of pulmonary fibrosis; Respir.Res., 17 103 4) Li et al. (2016), Oral pirfenidone protects against fibrosis by inhibiting fibroblast proliferation and TGF-b signaling in a murine colitis model; Biochem.Pharmacol., 117 57 5) Nakazato et al. (2002), A novel anti-fibrotic agent pirfenidone suppresses tumor necrosis factor-alpha at the translational level, Eur.J.Pharmacol. 446 177 6) Misra and Rabideau (2000), Pirfenidone inhibits NADPH-dependent microsomal lipid peroxidation and scavenges hydroxyl radicals, Mol.Cell Biochem. 204 119 7) Canestaro et al. (2016), Drug Treatment of Idiopathic Pulmonary Fibrosis: Systemic Review and Network Meta-Analysis; Chest, 149 756
RT
PATHWAY: Redox; TGF-beta; TNF; Proliferation; Cytokine -- RESEARCH AREA: Immunology -- DISEASE AREA: Inflammation