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Scleroderma pathogenesis: a pivotal role for fibroblasts as effector cells.

Abstract：

:Scleroderma (systemic sclerosis; SSc) is characterised by fibrosis of the skin and internal organs in the context of autoimmunity and vascular perturbation. Overproduction of extracellular matrix components and loss of specialised epithelial structures are analogous to the process of scar formation after tissue injury. Fibroblasts are the resident cells of connective tissue that become activated at sites of damage and are likely to be important effector cells in SSc. Differentiation into myofibroblasts is a hallmark process, although the mechanisms and cellular origins of this important fibroblastic cell are still unclear. This article reviews fibroblast biology in the context of SSc and highlights the potentially important place of fibroblast effector cells in fibrosis. Moreover, the heterogeneity of fibroblast properties, multiplicity of regulatory pathways and diversity of origin for myofibroblasts may underpin clinical diversity in SSc, and provide novel avenues for targeted therapy.

journal_name

Arthritis Res Ther

journal_title

Arthritis research & therapy

authors

Gilbane AJ,Denton CP,Holmes AM

doi

10.1186/ar4230

subject

Has Abstract

pub_date

2013-01-01 00:00:00

pages

215

issue

3

eissn

1478-6354

issn

1478-6362

pii

ar4230

journal_volume

15

pub_type

杂志文章,评审

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