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Rheumatology 2008 47(Supplement 5):v8-v9; doi:10.1093/rheumatology/ken278
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

This article appears in the following Rheumatology issue: Update in systemic sclerosis [View the issue table of contents]

Connective tissue growth factor: growth factor, matricellular organizer, fibrotic biomarker or molecular target for anti-fibrotic therapy in SSc?

D. Abraham1

1Division of Medicine, Research Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College Medical School, University College London, London, UK.

Correspondence to: D. Abraham, Division of Medicine, Research Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College Medical School, University College London, Royal Free Campus, Roland Hill Street, London NW3 PF, UK. E-mail: d.abraham{at}medsch.ucl.ac.uk


  Abstract

SSc is characterized by enhanced extracellular matrix (ECM) production resulting in excessive scarring and replacement fibrosis affecting the interstitial and vascular compartments of multiple organs. Although the precise molecular mechanisms driving fibrosis remain elusive, TGF-β and connective tissue growth factor (CTGF), are considered key mediators. CTGF is over-expressed in lesional tissue and enhanced levels in the circulation are an indicator of disease extent and severity. Rapidly induced by TGF-β and ET-1, CTGF activates several signal transduction pathways via surface receptors that modulate the functional activities of fibroblasts, endothelial and smooth muscle cells. In vivo, over-expression of CTGF causes ECM accumulation and promotes tissue fibrosis. In animal models of SSc, neutralization of CTGF with antibody blockade or siRNA, suppresses fibrogenesis. This article examines the role of CTGF as an integrator of extracellular signals, fibrotic biomarker and discusses the potential value of CTGF antagonism as a therapeutic strategy in SSc.

KEY WORDS: Connective tissue growth factor, The CCN family, Signal transduction, Matricellular regulatory molecule, Wound healing, Fibrosis, Tissue remodelling, Transforming growth factor-β, Fibroblast, Systemic sclerosis

Submitted 30 April 2008; Accepted 19 June 2008


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