This article is part of the supplement: Proceedings of Fibroproliferative disorders: from biochemical analysis to targeted therapies
Mechanisms of pulmonary fibrosis: role of activated myofibroblasts and NADPH oxidase
Division of Pulmonary, Allergy and Critical Care Medicine, University of Alabama at Birmingham, 1530 3rd Avenue South, THT 422, Birmingham, AL 35294-0006, USA
Fibrogenesis & Tissue Repair 2012, 5(Suppl 1):S23 doi:10.1186/1755-1536-5-S1-S23Published: 6 June 2012
A common feature of pathological fibrosis involving the lung and other organs is the persistent activation of myofibroblasts in injured tissues. Recent evidence supports the role of a member of the NADPH oxidase (NOX) gene family, NOX4, in myofibroblast differentiation, matrix synthesis and contractility. Additionally, NOX4 may contribute directly or indirectly to alveolar epithelial cell death, while myofibroblasts themselves acquire an apoptosis-resistant phenotype. Thus, NOX4 may be responsible for the cardinal features of progressive fibrosis - myofibroblast activation and epithelial cell dysrepair. Therapeutic targeting of NOX4 is likely to be effective in progressive cases of fibrosis involving multiple organs.