Hexose uptake and transport in polymorphonuclear leukocytes from patients with glycogen storage disease Ib

Ruth Potashnik, Arie Moran, Shimon W. Moses, Niza Peleg, Nava Bashan

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19 Scopus citations

Abstract

Neutrophil functions and glucose metabolism are known to be impaired in glycogen storage disease (GSD) lb patients. The uptake of nonmetabolizing glucose analogues into polymorphonuclear leukocytes (PMN) of GSD lb patients was studied. 2-Deoxyglucose (2-DOG) and 3-O-methylglucose are transported across the cell membrane by facilitated diffusion mediated by the glucose transporter. Because 2-DOG is phosphorylated within the cell, its uptake rate reflects hexose transport as long as phosphorylation is not rate-limiting. These conditions prevail only at low 2-DOG concentrations. Transport of 5 µM DOG into GSD lb patient PMN was found to be similar to controls (4.3 ± 0.5 and 4.65 ± 1.77 pmol/min × 106, respectively). In contrast, 2-DOG uptake at high concentrations (2 mM) decreased by 70% in patient PMN compared with control cells (0.17 ± 0.06 and 0.51 ± 0.11 nmol/min × 106, for patients and controls, respectively). Transport of 3-O-methylglucose (a glucose analogue that does not undergo intracellular phosphorylation) was not different in patient PMN compared with controls (1.86 ± 033 and 2.19 ± 0.30 nmol/min × 106, respectively). Hexose monophosphate shunt activity in PMN of GSD Ib patients at a glucose concentration of 2 mM was 43% of control values, whereas at 10 µM it was similar to controls. Taken together, these results suggest that the defect in glucose uptake and metabolism found in GSD lb patient PMN is due to an impairment in hexose phosphorylation rather than in a reduction in the transmembrane glucose transport activity.

Original languageEnglish
Pages (from-to)19-20
Number of pages2
JournalPediatric Research
Volume28
Issue number1
DOIs
StatePublished - 1 Jan 1990

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