The increase of glycosaminoglycan synthesis and accumulation on the cell surface of cultured skin fibroblasts in Werner's syndrome.

Glycosaminoglycans (GAGS) synthesis and accumulation on the cell surface of skin fibroblasts in two patients with Werner's syndrome were measured by the incorporation of 3H-glucosamine and 35S-sulfate. The strains of fibroblasts from Werner's syndrome (WF) had a much higher production of GAGS and accumulation on the cell surface than did the controls. Uronic acid was measured by means of the carbazole sulfuric acid method and orcinol method and it was revealed that WF fibroblasts had a large amount of total uronic acid in the cell layer, the largest portion being the iduronic acid fraction. Electrophoretic analysis of the GAGS of WF showed an increase of production. Increased release into the medium and accumulation of pericellular glycosaminoglycans was observed in hyaluronic acid (HA), heparan sulfate + dermatan sulfate (HS + DS), and chondroitin sulfate (CHS) fractions. No specific accumulation of GAGS in the intracellular pools was observed.