| First Author | Kim SY | Year | 2007 |
| Journal | FEBS Lett | Volume | 581 |
| Issue | 20 | Pages | 3833-8 |
| PubMed ID | 17659284 | Mgi Jnum | J:123718 |
| Mgi Id | MGI:3719326 | Doi | 10.1016/j.febslet.2007.07.013 |
| Citation | Kim SY, et al. (2007) Neutrophilia and elevated serum cytokines are implicated in glycogen storage disease type Ia. FEBS Lett 581(20):3833-8 |
| abstractText | Glycogen storage disease type Ia (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. |
