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Idiopathic Pulmonary Fibrosis (IPF) is a fatal fibrotic lung disease with a dismal prognosis. IPF is characterized by the accumulation of myofibroblasts and extracellular matrix proteins and subsequent destruction of lung architecture leading to declining lung function. Developmental pathways typically suppressed in adult lung tissue are aberrantly activated in IPF, yet not well understood. SOX9 is a member of the HMG-box family of transcription factors that are selectively expressed by epithelial progenitor cells. Specifically, SOX9 modulates branching morphogenesis in the lung, and the organized deposition of collagen, as a part of cartilage formation in multiple organs. Here, we hypothesized that the SOX9 is activated in distal lung mesenchymal cells and functions as a positive regulator of pulmonary fibrosis in IPF. To determine whether SOX9 is upregulated in distal mesenchymal cells of IPF …