Abstract
Phagocyte (neutrophil and monocyte) function was evaluated in a boy with glycogen storage disease type Ib. Neutrophils were found to be defective in motility and respiratory burst and monocytes showed a defect in respiratory burst but not in motility. These results suggested that the glucose-6-phosphate transport system plays a role in the function of neutrophils and monocytes and that these two phagocytes are different from each other in their energy metabolism for motility.
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Abbreviations
- GSD Ia:
-
Glycogen storage disease type Ia
- GSD Ib:
-
glycogen storage disease type Ib
- G6P:
-
glucose-6-phosphate
- G6Pase:
-
glucose-6-phosphatase
- PHA:
-
phytohemagglutinin
- CL:
-
chemiluminescence
- ZAS:
-
zymosan-activated serum
- FMLP:
-
N-formylmethionyl-leucyl-phenylalanine
- CSA:
-
colony-stimulating activity
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Ueno, N., Tomita, M., Ariga, T. et al. Impaired monocyte function in glycogen storage disease type Ib. Eur J Pediatr 145, 312–314 (1986). https://doi.org/10.1007/BF00439409
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DOI: https://doi.org/10.1007/BF00439409