RESEARCH ARTICLE

Getting Out of a Sticky Situation: Targeting the Myofibroblast in Scleroderma

Andrew Leask, * Open Modal Authors Info & Affiliations
The Open Rheumatology Journal 15 June 2012 RESEARCH ARTICLE DOI: 10.2174/1874312901206010163

Abstract

There is no treatment for the fibrosis observed in scleroderma (systemic sclerosis, SSc). Although genome-wide expression profiling has suggested that differences in gene expression patters between non-lesional and lesional skin are minimal, phenotypically these areas of tissue are quite different. In fact, lesional areas of scleroderma patients can be distinguished by the presence of a differentiated form of fibroblast, termed the myofibroblast. This cell type expresses the highly contractile protein α-smooth muscle actin (α-SMA). Fibroblasts isolated from SSc lesions excessively synthesize, adhere to and contract extracellular matrix (ECM) and display activated adhesive signaling pathways. Strategies aimed at blocking myofibroblast differentiation, persistence and activity are therefore likely to be useful in alleviating the fibrosis in scleroderma.

Keywords: PDGF, TGFβ, endothelin, rac, PPARγ, Smad1, pericyte, egr-1, CCN2, CTGF, PKCε..
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