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Role of CD248 as a potential severity marker in idiopathic pulmonary fibrosis

Lookup NU author(s): Dr Lee Borthwick, Professor Andrew FisherORCiD, Professor Christopher Buckley



This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


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 < 0.01). CD248 protein levels were significantly greater in IPF-derived lung fibroblasts vs normal lung fibroblasts (p < 0.01) and CD248 silencing significantly reduced the proliferation of lung fibroblasts, but did not affected myofibroblast differentiation.Conclusion: We conclude that CD248 overexpression is possibly involved in the pathogenesis of IPF and it has potential as a disease severity marker. Given that CD248 ligands are collagen type I, IV and fibronectin, we hypothesise that CD248 signalling represents a novel matrix-fibroblast interaction that may be a potential therapeutic target in IPF.

Publication metadata

Author(s): Bartis D, Crowley LE, D'Souza VK, Borthwick L, Fisher AJ, Croft AP, Pongracz JE, Thompson R, Langman G, Buckley CD, Thickett DR

Publication type: Article

Publication status: Published

Journal: BMC Pulmonary Medicine

Year: 2016

Volume: 16

Online publication date: 14/04/2016

Acceptance date: 23/03/2016

Date deposited: 08/07/2016

ISSN (electronic): 1471-2466

Publisher: BioMed Central Ltd


DOI: 10.1186/s12890-016-0211-7


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Funder referenceFunder name
European Union
State of Hungary
Wellcome trust
ERS-LTRF 2011-131ERS long term training fellowship
FP7-PEOPLE-IEF 300371FP7-Marie Curie Intra-European Fellowship
TAMOP4.2.4.A/2-11/1-2012-0001European Social Fund