Abstract
Fibroblasts cultured from patients with Marfan's disease show metachromasia with toluidine blue and accumulate increased amounts of glycosaminoglycan (GAG). Compared to fibroblasts from controls, more of the newly synthesized GAG is hyaluronic acid. Cycloheximide has a modest inhibiting effect on GAG accumulation compared to protein inhibition while serum depletion has a greater effect on inhibiting GAG accumulation than on reducing synthesis of new protein. Exogenous hyaluronic acid restores new accumulation of hyaluronic acid in serum depleted Marfan-derived cultures towards baseline while having almost no effect on cultures derived from controls. The effect is specific for hyaluronic acid as chondroitin sulfate or dextran sulfate are not stimulatory and is not due to stimulation of new protein synthesis.
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