Characterization of dermatan sulfate in mucopolysaccharidosis VI. Evidence for the absence of hyaluronidase-like enzymes in human skin fibroblasts.
Dermatan sulfate-chondroitin sulfate copolymers with a high content of dermatan sulfate are stored in cultured human skin fibroblasts from patients affected with mucopolysaccharidosis VI (Maroteaux-Lamy disease). Characterization of the storage material provided evidence that hyaluronidase-like enzymes are not present in these fibroblasts. This is based on the following observations: (i) dermatan sulfate chains stored intracellularly show no reduction of molecular size as compared with intact chains isolated from the extra-cellular space; (ii) the stored dermatan sulfate chains lack reducible end groups generated by endoglycosidases; (iii) homogenates of human skin fibroblasts do not degrade hyaluronate and (iv) the stored dermatan sulfate chains are degraded by testes hyaluronidase.
D002478:Cells, Cultured; D002807:Chondroitin / Q000031:analogs & derivatives* / Q000378:metabolism; D003871:Dermatan Sulfate / Q000378:metabolism*; D005347:Fibroblasts / Q000378:metabolism; D006025:Glycosaminoglycans / Q000378:metabolism; D006801:Humans; D006821:Hyaluronoglucosaminidase / Q000378:metabolism*; D008970:Molecular Weight; D009083:Mucopolysaccharidoses / Q000378:metabolism*; D009087:Mucopolysaccharidosis VI / Q000378:metabolism*; D012867:Skin / Q000201:enzymology / Q000378:metabolism*
D006025:Glycosaminoglycans; D003871:Dermatan Sulfate; D002807:Chondroitin; D006821:Hyaluronoglucosaminidase