抄録
KIMURA, A., HAYASHI, S., KOSEKI, M. and TSURUMI, K. Characteristics of Urinary Glycosaminoglycans Excreted by a Patient with the Hurler-Scheie Compound Syndrome. Tohoku J. exp. Med., 1982, 136 (1), 61-66 Glycosaminoglycan isolated from urine of a patient with the Hurler-Scheie compound syndrome consisted of dermatan sulfate (60%), heparan sulfate (34%) and chondroitin sulfate (6%). About 60% of both dermatan and chondroitin sulfates had molecular weight 8, 000-10, 000, while 95% of the heparan sulfate had molecular weight less than 6, 000. The total sulfate content of the glycosaminoglycans increased with decrease in molecular weight. N-sulfate content in the heparan sulfate, however, had no relation to molecular weight, and was 0.33 mole per mole of glucosamine on the average. About 70% of the heparan sulfate with the lowest molecular weight (1, 500) were composed of three repeating disaccharide units of heparan sulfate and two acetyl, one N-sulfate and three O-sulfate groups linked to the units. The dermatan sulfate contained 1.0-1.2 moles of sulfate per mole of galactosamine. Of the excess sulfate 45-65% were bound to iduronate residues and the rest to C-6 of N-acetylgalactosamine 4-sulfate residues. Most of the dermatan sulfate (83.2-100%) had nonsulfated iduronic acid at the non-reducing end. This finding is consistent with the defect of iduronidase in this disease.
