Altered collagen and glycosaminoglycan syntheses in familial scleroderma skin fibroblasts.

BACKGROUND

The main clinical symptoms of systemic scleroderma are caused by an accumulation of collagen in the tissues. Familial scleroderma is a very rare disorder in which systemic scleroderma occurs in the members of the same family.

OBJECTIVE

To know whether or not the biosynthetic properties of familial scleroderma skin fibroblasts are similar to those of nonfamilial scleroderma fibroblasts, syntheses of extracellular matrix components in the two fibroblast strains obtained from familial scleroderma patients were studied.

METHODS

Expression of collagen and glycosaminoglycans by cultured familial scleroderma fibroblasts was determined.

RESULTS

Collagen synthesis by patient fibroblasts was elevated 1.4-fold compared with control fibroblasts. Translational activity of type I procollagen mRNA and the level of alpha 1 (I) mRNA in the patient fibroblasts was increased 1.7- and 1.6-fold, respectively. The ratio of hyaluronic acid/dermatan sulfate in the patient fibroblasts was lower than that in control fibroblasts.

CONCLUSION

The results indicate that the biosynthetic phenotypes of familial scleroderma fibroblasts are similar to those of nonfamilial scleroderma fibroblasts which have already been reported.