Abstract
During repair of connective tissue, resting fibroblasts become ‘activated’; that is, they migrate into the wound where they synthesize and remodel new extracellular matrix. The differentiated fibroblast responsible for this action is termed the myofibroblast, which expresses the highly contractile protein α—smooth muscle actin (α—SMA) and is responsible for the synthesis and remodeling of extracellular matrix (ECM). Persistence of the myofibroblast is a key characteristic of fibrotic diseases including scleroderma. Proteins such as transforming growth factorβ (TGFβ), endothelin-1 (ET- 1), connective tissue growth factor (CCN2/CTGF) and platelet derived growth factor (PDGF) are believed to contribute to myofibroblast differentiation and persistence. Moreover, it is now known that elevated adhesive and contractile signaling is a key feature of fibrotic fibroblasts. This review summarizes recent findings aimed at developing new, rationally-designed therapies for the fibrosis in scleroderma.
Current Enzyme Inhibition
Title: Sticking it to Scleroderma: Potential Therapies Blocking Elevated Adhesive and Contractile Signaling
Volume: 6 Issue: 2
Author(s): Andrew Leask
Affiliation:
Keywords: PDGF, TGFβ, endothelin, rac, PPARγ, PKCε
Abstract: During repair of connective tissue, resting fibroblasts become ‘activated’; that is, they migrate into the wound where they synthesize and remodel new extracellular matrix. The differentiated fibroblast responsible for this action is termed the myofibroblast, which expresses the highly contractile protein α—smooth muscle actin (α—SMA) and is responsible for the synthesis and remodeling of extracellular matrix (ECM). Persistence of the myofibroblast is a key characteristic of fibrotic diseases including scleroderma. Proteins such as transforming growth factorβ (TGFβ), endothelin-1 (ET- 1), connective tissue growth factor (CCN2/CTGF) and platelet derived growth factor (PDGF) are believed to contribute to myofibroblast differentiation and persistence. Moreover, it is now known that elevated adhesive and contractile signaling is a key feature of fibrotic fibroblasts. This review summarizes recent findings aimed at developing new, rationally-designed therapies for the fibrosis in scleroderma.
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Cite this article as:
Leask Andrew, Sticking it to Scleroderma: Potential Therapies Blocking Elevated Adhesive and Contractile Signaling, Current Enzyme Inhibition 2010; 6 (2) . https://dx.doi.org/10.2174/157340810791232999
DOI https://dx.doi.org/10.2174/157340810791232999 |
Print ISSN 1573-4080 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6662 |
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