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 molecularweight 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.