IMR Press / FBE / Volume 1 / Issue 1 / DOI: 10.2741/E12

Frontiers in Bioscience-Elite (FBE) is published by IMR Press from Volume 13 Issue 2 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.


Signaling in fibrosis: targeting the TGFbeta, endothelin-1 and CCN2 axis in scleroderma

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1 CIHR Group in Skeletal Development and Remodeling, Division of Oral Biology, Department of Physiology and Pharmacology, Schulich School of Medicine and Dentistry, University of Western Ontario, London ON, Canada
Front. Biosci. (Elite Ed) 2009, 1(1), 115–122;
Published: 1 June 2009

Fibrosis affects organs such as the skin, liver, kidney and lung and is a cause of significant morbidity. There is no therapy for fibrosis. Recent significant molecular insights into the signaling underlying fibrosis have been made. Transforming growth factor beta (TGFbeta) signaling is a major contributor to fibrogenesis. The signaling mechanisms through which TGFbeta induces fibrogenic responses have been under intense scrutiny. Moreover, the potent pro-fibrotic proteins endothelin-1 (ET-1) and CCN2 (connective tissue growth factor, CTGF) are believed to play an essential role in this process as downstream regulators or co-factors of TGFbeta signaling. This review summarizes these recent crucial observations with emphasis on the disease scleroderma.

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