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BACKGROUND: CD248 or Endosialin is a transmembrane molecule expressed in stromal cells binding to extracellular matrix (ECM) components. It has been previously implicated in kidney fibrosis, rheumatoid arthritis as well as in tumour-stromal interactions. This study investigates the role of CD248 in the pathogenesis of fibrotic diseases in Idiopathic Pulmonary Fibrosis (IPF). METHODS: CD248 quantitative immunohistochemistry (IHC) was performed on lung samples from 22 IPF patients and its expression was assayed in cultured pulmonary fibroblasts and epithelial cells. Effects of CD248 silencing was evaluated on fibroblast proliferation and myofibroblast differentiation. RESULTS: IHC revealed strong CD248 expression in mesenchymal cells of normal lung structures such as pleura and adventitia but not in epithelium. Fibrotic areas showed markedly stronger staining than unaffected lung tissue. The extent of CD248 staining showed a significant negative correlation to lung function parameters FEV1, FVC, TLC, and TLCO (r2 > 0 · 35, p 

Original publication




Journal article


BMC Pulm Med

Publication Date





Biomarker, CD248, Endosialin, IPF, Idiopathic pulmonary fibrosis, TGF-beta, Adult, Aged, Antigens, CD, Antigens, Neoplasm, Biomarkers, Biopsy, Cell Proliferation, Cells, Cultured, Female, Fibroblasts, Flow Cytometry, Gene Expression Regulation, Humans, Idiopathic Pulmonary Fibrosis, Immunohistochemistry, Lung, Male, Middle Aged, RNA, Real-Time Polymerase Chain Reaction, Retrospective Studies, Severity of Illness Index, Signal Transduction