The role of epigenetic regulation in idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a serious respiratory disease with a poor prognosis and limited treatments. IPF is characterized by accumulation of extracellular matrix, destruction of gas exchange units, and decline in lung function. The pathogenesis and underlying mechanisms are poorly understood although a combination of environmental and genetic factors is implicated. Epigenetic modifications represent a facet of gene regulation likely important to key pathways including transforming growth factor (TGF-β1) signaling, fibroblast-to-myofibroblast differentiation, epithelial-to-mesenchymal transition, cellular aging, and apoptosis. Methylation, acetylation, noncoding RNAs, telomere length, and G-quadruplexes have all been investigated with varying levels of progress but are certainly potential targets for drug development. This review discusses the underlying epigenetic biology of IPF, associated clinical applications, and potential novel treatments.