Characterization of dermatan sulfate and heparan sulfate in the urine of a patient with the Hunter syndrome.
Glycosaminoglycan isolated from the urine of a patient with the Hunter syndrome was composed of heparan sulfate (59.9%), dermatan sulfate (30.6%) and chondroitin sulfate (9.5%), and was heterogeneous in molecular weight (1,500-10,000) and in sulfate content (0.35-2.05 moles/mole of hexosamine). About 60% of dermatan sulfate and 10% of heparan sulfate had molecular weight of 7,000 to 10,000, while about 10% of the former and 60% of the latter had those of 1,500 to 3,500. Sulfate contents of dermatan sulfate and heparan sulfate were inversely related to their molecular weights. Higher total- and N-sulfate contents were measured in smaller molecular-weight heparan sulfate, and higher acetyl content was in larger molecular-weight heparan sulfate. On the basis of the chemical properties of dermatan sulfate and heparan sulfate isolated in this experiment, their catabolic processes in the Hunter syndrome were discussed.
D000596:Amino Acids / Q000032:analysis; D002675:Child, Preschool; D002807:Chondroitin / Q000031:analogs & derivatives*; D003871:Dermatan Sulfate / Q000032:analysis / Q000652:urine*; D004586:Electrophoresis; D006025:Glycosaminoglycans / Q000652:urine*; D006497:Heparan Sulfate / Q000032:analysis / Q000652:urine*; D006801:Humans; D008297:Male; D008970:Molecular Weight; D016532:Mucopolysaccharidosis II / Q000652:urine*
D000596:Amino Acids; D002807:Chondroitin; D003871:Dermatan Sulfate; D006025:Glycosaminoglycans; D006497:Heparan Sulfate