The mighty fibroblast and its utility in scleroderma research

Abstract

Fibroblasts are the effector cells of fibrosis characteristic of systemic sclerosis (SSc, scleroderma) and other fibrosing conditions. The excess production of extracellular matrix (ECM) proteins is the hallmark of fibrosis in different organs, such as skin and lung. Experiments designed to assess the pro-fibrotic capacity of factors, their signaling pathways, and potential inhibitors of their effects that are conducted in fibroblasts have paved the way for planning clinical trials in SSc. As such, fibroblasts have proven to be valuable tools in the search for effective anti-fibrotic therapies for fibrosis. Herein we highlight the characteristics of fibroblasts, their role in the etiology of fibrosis, utility in experimental assays, and contribution to drug development and clinical trials in SSc.

Keywords: Extracellular matrix (ECM); Fibroblasts; Fibrosis; Scleroderma; Systemic sclerosis (SSc).

Fig. 1

Morphology of primary human lung fibroblasts grown on tissue culture-treated plastic, collagen-coated plastic, and in a 3D collagen matrix (PureCol). Cells were plated at equal densities and images were taken 24-h and 72-h post-seeding. Note that cells in PureCol have migrated within the 3D matrix and not all are visible within one focal plane.