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. 2012 Jun 6;5(Suppl 1):S23.
doi: 10.1186/1755-1536-5-S1-S23. eCollection 2012.

Mechanisms of pulmonary fibrosis: role of activated myofibroblasts and NADPH oxidase

Victor J Thannickal  1
Affiliations

Mechanisms of pulmonary fibrosis: role of activated myofibroblasts and NADPH oxidase

Victor J Thannickal. Fibrogenesis Tissue Repair. .

Abstract

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.

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Figures

Figure 1
Figure 1
Hypothetical model of a central role of NADPH oxidase-4 (NOX4) in pathological fibrosis: Aging, environmental and genetic/epigenetic factors influence the persistent expression/activation of NADPH oxidase 4 (NOX4) in fibrotic tissues. This may be due to aberrations in injury repair responses or in maintenance regeneration, resulting in a failure to maintain cellular homeostasis in fibrotic tissues. Loss of tissue homeostasis is characterized by myofibroblast activation and epithelial cell dysrepair, with their attendant cellular phenotypes, that promotes pathological tissue fibrosis.
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