Studies on the composition of urinary glycosaminoglycans and oligosaccharides in patients with mucopolysaccharidoses who were receiving fibroblast transplants.
This communication reports studies on the composition of the urinary glycosaminoglycans and oligosaccharides in mucopolysaccharidosis patients who were being treated by fibroblast transplantation. The urinary glycosaminoglycans were precipitated with 9-aminoacridine, the oligosaccharides remaining in solution. Both fractions were further subfractionated by gel filtration. The glycosaminoglycan subfractions were examined for their content of iduronic acid, glucuronic acid, galactosamine and glucosamine. We found no changes in these parameters in a patient who had been treated by repeated fibroblast transplantations over the course of 4 1/2 years. The amino sugar composition of the oligosaccharide fraction was examined and shown to be unchanged. We also found no changes in the degree of sulphation of the urinary glycosaminoglycans specifically related to the transplant in four patients with Hurler disease and two with Hunter disease. We conclude that fibroblast transplantation does not produce detectable changes in the carbohydrate content or degree of sulphation of the urinary glycosaminoglycans and oligosaccharides.
D002648:Child; D005260:Female; D005347:Fibroblasts / Q000637:transplantation*; D005688:Galactosamine / Q000032:analysis; D005944:Glucosamine / Q000032:analysis; D006025:Glycosaminoglycans / Q000652:urine*; D006801:Humans; D008297:Male; D009083:Mucopolysaccharidoses / Q000628:therapy / Q000652:urine*; D008059:Mucopolysaccharidosis I / Q000628:therapy / Q000652:urine; D016532:Mucopolysaccharidosis II / Q000628:therapy / Q000652:urine; D009844:Oligosaccharides / Q000652:urine*; D014574:Uronic Acids / Q000032:analysis
D006025:Glycosaminoglycans; D009844:Oligosaccharides; D014574:Uronic Acids; D005688:Galactosamine; D005944:Glucosamine