期刊
FEBS LETTERS
卷 581, 期 20, 页码 3833-3838出版社
WILEY
DOI: 10.1016/j.febslet.2007.07.013
关键词
glycogen storage disease type I; glucose-6-phosphatase-alpha; neutrophilia; granulocyte colony stimulating factor; cytokine-induced neutrophil chemoattractant; myeloid function
资金
- Intramural NIH HHS [Z01 HD000912-28] Funding Source: Medline
Glycogen storage disease type la (GSD-Ia) patients deficient in glucose-6-phosphatase-alpha manifest a disturbed glucose homeostasis. We hypothesized that disturbed glucose homeostasis might affect myeloid functions. Here, we show that GSD-Ia mice exhibit normal neutrophil activities but have elevated myeloid progenitor cells in the bone marrow and spleen. Interestingly, GSD-Ia mice exhibit a persistent increase in peripheral blood neutrophil counts along with elevated serum levels of granulocyte colony stimulating factor and cytokine-induced neutrophil chemoattractant. Taken together, our results suggest that a loss of glucose homeostasis can compromise the immune system, resulting in neutrophilia. This may explain some of the unexpected clinical manifestations seen in GSD-Ia. Published by Elsevier B.V. on behalf of the Federation of European Biochemical Societies.
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