A fibroblast mitogen present in scleroderma but not control sera: Inhibition by proteinase inhibitors

LeRoy, E. Carwile; Kahaleh, M. Bashar; Mercurio, S.

doi:10.1007/bf00541230

Cited by 18 publications

(1 citation statement)

References 8 publications

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“…This evidence for different subpopulations of fibroblasts in scleroderma skin prompted additional studies, and even in normal skin some demonstrated different clones of fibroblasts characterized by a high or low synthesis of collagen. In addition, when normal fibroblasts were grown in the presence of serum obtained from scleroderma patients these activated subclones were preferentially stimulated [30]. However, identification of these clones in vivo is difficult and there are only few attempts to develop markers recognizing distinct fibroblast subpopulations.…”

Section: Smentioning

confidence: 98%

Control of Fibrosis in Systemic Scleroderma.

Mauch¹,

Eckes²,

Hunzelmann³

et al. 1993

J Invest Dermatol

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Scleroderma is characterized by an excessive deposition of collagen in all involved organs. This is due to an overproduction of extracellular matrix (ECM) molecules following induction of gene expression, whereas there is no evidence that the composition of the connective tissue matrix is altered. Several in vivo studies and in vitro experiments suggest that a close interaction between inflammatory cells and fibroblasts is required for the initial activation of fibroblasts. TGF-beta presumably plays an important role, but other cytokines, e.g., PDGF or FGF, may also be involved. Many of the ECM molecules have been shown to interact closely with fibroblasts and provide signals that regulate fibroblast metabolism. The cellular response towards those signals is a further aspect of fibrosis that has attracted attention during recent years. The altered expression of receptor proteins on the cell surface of scleroderma fibroblasts for example might explain in part the lack of down-regulation of collagen synthesis in late phases of the disease. This review summarizes the alterations of connective tissue in scleroderma, and discusses the role of cytokines as well as the ECM for the regulation of fibroblast function and their implication for the development of fibrosis.

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Section: Smentioning

confidence: 98%