Chemical structure of urinary dermatan sulfate excreted by a patient with the Hunter syndrome.
The chemical structure of dermatan sulfate (DS) in the urine of a patient the Hunter syndrome was studied through the analysis of disaccharide units which were derived from the urinary DS by digestion with chondroitinase ABC and separated on a Dowex 1 column. The DS was basically composed of repeating disaccharide units of iduronyl N-acetylgalactosamine 4-sulfate. About 90% of the excess sulfate were linked to the iduronate residues as an additional sulfate group in the unit. N-Acetylgalactosamine 6-sulfate and N-acetylgalactosamine 4,6-disulfate residues were minor components. No non-sulfated disaccharide unit was detected in the digestion products. Only sulfoiduronate residue was found as the non-reducing terminal sugar of the DS molecule, consistent with the lack of iduronosulfate sulfatase in this disease.
D000116:Acetylgalactosamine / Q000032:analysis; D000818:Animals; D055598:Chemical Phenomena; D002621:Chemistry; D002675:Child, Preschool; D002807:Chondroitin / Q000031:analogs & derivatives*; D002809:Chondroitin Sulfates / Q000652:urine; D002810:Chondroitinases and Chondroitin Lyases / Q000494:pharmacology; D003871:Dermatan Sulfate / Q000032:analysis / Q000652:urine*; D004187:Disaccharides / Q000032:analysis; D006801:Humans; D008297:Male; D016532:Mucopolysaccharidosis II / Q000652:urine*; D012867:Skin / Q000032:analysis; D013552:Swine
D004187:Disaccharides; D003871:Dermatan Sulfate; D002807:Chondroitin; D002809:Chondroitin Sulfates; D002810:Chondroitinases and Chondroitin Lyases; D000116:Acetylgalactosamine