The Journal of Biochemistry
Online ISSN : 1756-2651
Print ISSN : 0021-924X
Neutral Oligosaccharides in the Urine of a Patient with Glycogen Storage Disease Type II
Masaru KURIYAMARyo-ichi HIWATARIToshio ARIGAYoshiyuki SAKANOJun-ichi ABEMitsuhiro OSAMEAkihiro IGATA
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JOURNAL FREE ACCESS

1985 Volume 98 Issue 4 Pages 1041-1047

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Abstract

Neutral oligosaccharides were isolated from urine of an adult patient with glycogen storage disease type II, a deficiency of lysosomal acid α-glucosidase, by chromatography on columns of activated charcoal, Dowex 50×2 and Dowex 1×2. Total neutral oligosaccharides in the urine of the patient were increased about 5-fold as compared with those in normal controls. The most accumulated oligosaccharide was separated by Bio-Gel P-2 column chromatography, and finally purified by paper chromatography. Based on various studies, including carbohydrate analysis, chemical ionization mass spectrometry, fast atom bombardment mass spectrometry, degradation by glucoamylase and isopullulanase, and methylation analysis, the structure of this oligosaccharide was deduced to be Glcα1→ 6Glcα1→4Glcα1→4Glc. This oligosaccharide appears to be accumulated in urine of the patient with acid α-glucosidase deficiency as an end product of the hydrolysis of glycogen.

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© The Japanese Biochemical Society
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