Abstract
KOSEKI, M., INO, S., KIMURA, A. and TSURUMI, K. Abnormal Urinary Excretionof Sialoglycoconjugates in Patients with Mucopolysaccharidosis. Tohoku J.exp. Med., 1981, 135 (4), 431-439-Abnormal urinary excretion of sialoglycoconjugateswas observed in four patients with mucopolysaccharidosis. Urinarysialoglycoconjugates were fractionated into 8 fractions by sequential gel filtrationon Sephadex G-25, G-50 and by Dowex 1 ion-exchange chromatography.The comparison of the amounts of these fractions indicated that the fraction richin mannose and glucosamine contents contributed to the increased urinary excretionof sialoglycoconjugates in patients with mucopolysaccharidosis. The majorcomponent of this fraction was disialyl-oroso-N-octaose which was a representativeoligosaccharide side chain of glycoproteins with an Asn-GlcNAc linkage. Undoubtedlythis abnormality is the secondary lesion of mucopolysaccharidosis, butit is conceivable that the disturbed metabolism of sialoglycoprotein is closely relatedto the pathogenesis of these diseases.-lysosomal storage diseases;mucopolysaccharidosis; sialyloligosaccharides; sialoglycoconjugates
