In vitro correction of Hurler fibroblasts with bovine testicular hyaluronidase.
Bovine testicular hyaluronidase (endo-beta-N-acetyl hexosaminidase) has a significant corrective effect on cultured Hurler fibroblasts. Nonspecificity of this effect is indicated by its equally strong corrective effect on Hunter fibroblasts. Although all specimens of hyaluronidase also possessed iduronidase activity, a separate corrective effect could be attributed to the endo-N-acetyl hexosaminidase activity of at least one hyaluronidase (Wyeth M-151) for four reasons: (i) its very low content of iduronidase activity; (ii) a decrease in intracellular macromolecular mucopolysaccharides (believed to be largely dermatan sulfate) with a corresponding increase in intracellular and extracellular oligosaccharides; (iii) no measurable increase in iduronidase activity of hyaluronidase-treated cells despite near maximal correction; (iv) direct correlation between Hurler cell correction and hyaluronidase activity when enzymes of different strength were used at less than maximal correction.
D000818:Animals; D002417:Cattle; D002478:Cells, Cultured; D006025:Glycosaminoglycans / Q000378:metabolism*; D006801:Humans; D006821:Hyaluronoglucosaminidase / Q000378:metabolism / Q000494:pharmacology*; D007066:Iduronate Sulfatase / Q000378:metabolism; D008297:Male; D008059:Mucopolysaccharidosis I / Q000378:metabolism*; D016532:Mucopolysaccharidosis II / Q000378:metabolism*; D010447:Peptide Hydrolases / Q000494:pharmacology; D013737:Testis / Q000201:enzymology
D006025:Glycosaminoglycans; D007066:Iduronate Sulfatase; D006821:Hyaluronoglucosaminidase; D010447:Peptide Hydrolases