Literature DB >> 3168292

Urinary excretion of a glucose-containing tetrasaccharide. A parameter for increased degradation of glycogen.

J Kumlien1, M A Chester, B S Lindberg, P Pizzo, D Zopf, A Lundblad.   

Abstract

The urinary excretion of a glucose-containing oligosaccharide, Glc alpha[1-6Glc alpha[1-4Glc alpha[1-4Glc, (Glc4) has been measured in various physiological and pathological conditions. The Glc4 content of 24 h samples from the same individual was relatively constant, whereas 2 h samples showed up to 4-fold variations in Glc4 concentration. This variation is associated mainly with increased excretion of Glc4 after meals. A carbohydrate-rich diet, starvation or a protein-rich diet, and intense physical activity all affected the urinary excretion of Glc4. Both oral and intravenous administration of glycogen in a Rhesus monkey resulted in increased excretion of Glc4. When Glc4 itself was injected intravenously in small amounts renal clearance was rapid and complete. In contrast, injection of a larger amount resulted in incomplete (approximately 10%) renal clearance, probably due to uptake and metabolism of the oligosaccharide. In patients with glycogen storage diseases, certain malignancies, and pancreatitis, 24 h urinary Glc4 excretion exceeded the normal range. The diagnostic implications of these observations deserve evaluation. The results presented suggest a need for standardization of nutritional status and physical activity when monitoring urinary Glc4 excretion for diagnostic purposes.

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Year:  1988        PMID: 3168292     DOI: 10.1016/0009-8981(88)90172-6

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN: 0009-8981            Impact factor:   3.786


  5 in total

1.  Hepatic glycogenosis with defects in the glycogen breakdown pathway: urinary oligosaccharide profile.

Authors:  M L Giros; L Alvarez
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982

2.  Baseline Urinary Glucose Tetrasaccharide Concentrations in Patients with Infantile- and Late-Onset Pompe Disease Identified by Newborn Screening.

Authors:  Yin-Hsiu Chien; Jennifer L Goldstein; Wuh-Liang Hwu; P Brian Smith; Ni-Chung Lee; Shu-Chuan Chiang; Adviye A Tolun; Haoyue Zhang; Amie E Vaisnins; David S Millington; Priya S Kishnani; Sarah P Young
Journal:  JIMD Rep       Date:  2015-02-15

3.  Determination of a glucose-containing tetrasaccharide in urine of patients with acute pancreatitis.

Authors:  J Kumlien; A Andrén-Sandberg; D Zopf; A Lundblad
Journal:  Int J Pancreatol       Date:  1989-03

4.  Diurnal variability of glucose tetrasaccharide (Glc4) excretion in patients with glycogen storage disease type III.

Authors:  Sarah P Young; Aleena Khan; Ela Stefanescu; Andrea M Seifts; Ghada Hijazi; Stephanie Austin; Priya S Kishnani
Journal:  JIMD Rep       Date:  2020-11-03

5.  Experience with the Urinary Tetrasaccharide Metabolite for Pompe Disease in the Diagnostic Laboratory.

Authors:  Jennifer T Saville; Maria Fuller
Journal:  Metabolites       Date:  2021-07-08
  5 in total

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