Synthesis and Degradation of Hyaluronic Acid in the Cultured Fibroblasts of Marfan's Disease

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Abstract

Increased amounts of hyaluronic acid accumulate in fibroblasts cultured from patients with Marfan's disease, an autosomal dominant disorder. In the recessive Hurler's disease, the storage of glycosaminoglycan (GAG) is due to impaired degradation. This study examines the kinetics of GAG accumulation in Marfan's disease in order to determine whether the mechanism of accumulation differs from that in Hurler's disease.

Authors

Stanford I. Lamberg, Albert Dorfman