Deciphering the pro-fibrotic phenotype of fibroblasts in systemic sclerosis
- PMID: 24476007
- DOI: 10.1111/exd.12237
Deciphering the pro-fibrotic phenotype of fibroblasts in systemic sclerosis
Abstract
LeRoy's seminal work on the phenotypic features of scleroderma fibroblasts has been directing fibrosis research in the field of systemic sclerosis (SSc, scleroderma) for the past 30 years. His principal experiment, to culture skin fibroblasts from patients with SSc and study their pro-fibrotic phenotype in comparison with skin fibroblasts from healthy individuals, has been used by most basic and translational fibrosis studies in SSc. LeRoy's findings have revolutionized our understanding of the disease pathogenesis and guided the development of novel antifibrotic therapies towards fibroblast-specific approaches.
Keywords: fibroblast; fibrosis; pathogenesis; systemic sclerosis; treatment.
© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
Comment on
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Connective tissue synthesis by scleroderma skin fibroblasts in cell culture.J Exp Med. 1972 Jun 1;135(6):1351-62. doi: 10.1084/jem.135.6.1351. J Exp Med. 1972. PMID: 4260235 Free PMC article.
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Increased collagen synthesis by scleroderma skin fibroblasts in vitro: a possible defect in the regulation or activation of the scleroderma fibroblast.J Clin Invest. 1974 Oct;54(4):880-9. doi: 10.1172/JCI107827. J Clin Invest. 1974. PMID: 4430718 Free PMC article.
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