Alterations in Synthesis of Various Connective Tissue Components in Werner’s Syndrome Cultured Dermal Fibroblasts
Werner's syndrome (WS), a premature aging disorder, is known to have scleroderma-like skin changes. We studied both collagen and glycosaminoglycan (GAG) synthesis in dermal fibroblasts derived from two patients with WS. The total synthesized protein was determined by the incorporation of [3H]-proline, and the synthesized collagen was measured as labeled hydroxyproline. The GAG synthesis was measured by the incorporation of [3H]-glucosamine. The synthesis of total and collagenous protein of fibroblasts from WS was increased about threefold respective to controls. The GAG synthesis of fibroblasts from WS patients was also significantly higher than normal controls. These results suggest that the connective tissue synthesis in WS might be common to be that of scleroderma in the active phase.
