Molecular aspects of scleroderma

被引：41

作者：

Trojanowska, M ^{[1
]}

机构：

[1] Med Univ S Carolina, Div Rheumatol & Immunol, Charleston, SC 29425 USA

来源：

FRONTIERS IN BIOSCIENCE-LANDMARK | 2002年 / 7卷

关键词：

scleroderma; TGF-beta; CTGF; collagen; fibrosis; review;

D O I：

10.2741/trojan

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页码：D608 / D618

页数：11

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