Proteoglycans in cultures of skin fibroblasts in recessive dystrophic epidermolysis bullosa.

The proteoglycans of cultured fibroblasts from the skin of three patients with recessive dystrophic epidermolysis bullosa and three normals were compared after labeling with [35S]sulfate and [3H]leucine. The behavior in gel chromatography of the intact proteoglycans and several properties of their component glycosaminoglycans (size, content of iduronic acid, and content of 4- and 6-sulfate) showed no statistical differences. In addition, the binding of intact proteoglycans and of their constituent proteins and glycosaminoglycans to type I collagen were measured by affinity chromatography. No differences were found that could account for the skin lesions in recessive dystrophic epidermolysis bullosa.