Deciphering the pro-fibrotic phenotype of fibroblasts in systemic sclerosis

Exp Dermatol. 2014 Feb;23(2):99-100. doi: 10.1111/exd.12237.

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.

Publication types

  • Comment

MeSH terms

  • Collagen / biosynthesis*
  • Fibroblasts / metabolism*
  • Glycoproteins / biosynthesis*
  • Humans
  • Scleroderma, Systemic / metabolism*
  • Skin / metabolism*

Substances

  • Glycoproteins
  • Collagen