IMR Press / FBL / Volume 7 / Issue 4 / DOI: 10.2741/trojan

Frontiers in Bioscience-Landmark (FBL) is published by IMR Press from Volume 26 Issue 5 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with Frontiers in Bioscience.

Open Access Article
Molecular aspects of scleroderma
Show Less
1 Division of Rheumatology and Immunology, Medical University of South Carolina, 96 Jonathan Lucas St., Suite 912, Charleston SC 29425, USA
Academic Editor:Marcos Rojkind
Front. Biosci. (Landmark Ed) 2002, 7(4), 608–618; https://doi.org/10.2741/trojan
Published: 1 March 2002
(This article belongs to the Special Issue From regeneration to scar formation)
Abstract

Scleroderma is a complex disease characterized by activation of the immune system, small-vessel vasculopathy, and fibrosis of the skin and other organs. This review is limited to the discussion of scleroderma fibroblast biology and the cellular and molecular mechanisms that contribute to the abnormal deposition of collagen. Selected aspects of abnormal extracellular matrix (ECM) regulation by scleroderma fibroblasts are critically reviewed. These include the role of transforming growth factor-beta (TGF-beta) and connective tissue growth factor (CTGF) and their receptors in the fibrotic process in scleroderma and the overview of the transcription factors involved in regulation of the human alpha2 (I) collagen (COL1A2) gene.

Keywords
Scleroderma
TGF-beta
CTGF
collagen
fibrosis
Review
Share
Back to top