Abstract
Abstract The qualitative and quantitative analyses of urinary chondroitin sulfates in patients with Hurler's Hunter's and Maroteaux-Lamy's syndromes were carried out in comparison with those of a normal subject on the basis of disaccharide subunit levels after digestion of the chondroitin sulfates with chondroitinases and chondrosulfatases. The minor quantities of unsaturated 4-sulfated and 6-sulfated disaccharides were detected in the degraded production of urinary chondroitin sulfates in these patients after digestion with chondroitinase-AC. A striking increase of unsaturated 4-sulfated disaccharide derived from chondrotin sulfate B was shown after degradation with chondroitinase-ABC. The minorities of unsaturated non-sulfated and di-sulfated disaccharides were also detected as disaccharide chains of the isomers. The findings indicate that minor constituents of chondroitin sulfate A and C are present in addition to the majority of chondroitin sulfate B in the urinary chondroitin sulfate group in these syndromes. The presence of minor quantities of the disaccharide subunits of non- and/or undersulfated chondroitin sulfate and oversulfated chondroitin sulfate isomers in the syndromes.
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