Patients with glycogen storage disease type I1 and type I11 were recently found to excrete increased amounts of a glucose-containing tetrasaccharide ~G l~p (~l -+ 6 )~G l c p ( c r l -t 4 )~G l c p (~l -+ 4 )~G l c [Lennartson, G., Lundblad, A., Sjoblad, S., Svensson, S. and Ockerman,P. A. (1976) Biomed. Muss Spectrom. 3,51-541. In addition to this tetrasaccharide, urine from these patients also contains larger oligosaccharides containing only glucose. From urine of patients with glycogen storage disease type I1 and type 111, three and four oligosaccharides respectively have been isolated. Structural studies including sugar analyses, methylation analyses, partial acid hydrolysis and optical rotation revealed that three compounds were present in the urine of both patients. Their proposed structures or partial structures are as follows: ~Glcp(ct1-6)~Glcp(zl-6)~Glcp (cnl-4)
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