Neutrophilia and elevated serum cytokines are implicated in glycogen storage disease type Ia

FEBS Lett. 2007 Aug 7;581(20):3833-8. doi: 10.1016/j.febslet.2007.07.013. Epub 2007 Jul 16.


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.

Publication types

  • Research Support, N.I.H., Intramural

MeSH terms

  • Animals
  • Calcium / analysis
  • Calcium / metabolism
  • Chemokine CXCL2
  • Chemotaxis, Leukocyte / drug effects
  • Chemotaxis, Leukocyte / physiology
  • Cytokines / blood*
  • Glycogen Storage Disease Type I / etiology*
  • Glycogen Storage Disease Type I / metabolism*
  • Glycogen Storage Disease Type I / pathology
  • Mice
  • Mice, Knockout
  • Monokines / pharmacology
  • N-Formylmethionine Leucyl-Phenylalanine / pharmacology
  • Neutrophils / metabolism*
  • Respiratory Burst / physiology


  • Chemokine CXCL2
  • Cytokines
  • Monokines
  • N-Formylmethionine Leucyl-Phenylalanine
  • Calcium