Open AccessGlucose‐Containing Oligosaccharides in the Urine of Patients with Glycogen Storage Disease Type II and Type III
Author(s) -
LENNARTSON Gudrun,
LUNDBLAD Arne,
LUNDSTEN Jörgen,
SVENSSON Sigfrid,
HÄGER Anders
Publication year - 1978
Publication title -
european journal of biochemistry
Language(s) - English
Resource type - Journals
eISSN - 1432-1033
pISSN - 0014-2956
DOI - 10.1111/j.1432-1033.1978.tb12098.x
Subject(s) - tetrasaccharide , chemistry , glycogen , urine , biochemistry , carbohydrate , hydrolysis , polysaccharide
Patients with glycogen storage disease type II and type III were recently found to excrete increased amounts of a glucose‐containing tetrasaccharide d Gle p (α→ 6) d Glc p (α→ 4) d Gle p (α1 → 4) d Glc [Lennartson, G., Lundblad, A., Sjöblad, S., Svensson,S. and Öckerman,P.A.(1976) Biomed. Mass Spectrom. 3 , 51–54]. 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 II and type III, 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: d Glc p (α1–6) d Glc p (α1–4) d Glc p (α1–4) d Glc p (α1–4) d Glc, d Glc p (α1–4) d Glc p (α1–6) d Glc p (α1–6) d Glc p (α1–4) d Glc p (α1–4) d Glc, and d Glc p ‐(α1–6) d Glc p (α1–4) d Glc p (α1–4) d Glc p (α1–4) d Glc p (α1–6) d Glc p (α1–4) d Glc p (α1–4) d Glc. A fourth compound has been partially characterized as a branched heptasaccharide with four (1→4) linkages and two (1→6) linkages. Glycogen is possibly the origin of these compounds. However, the number of (1→6) linkages is higher than expected and may indicate a shorter distance between branches in glycogen than has been generally assumed.