Neutrophilia and elevated serum cytokines are implicated in glycogen storage disease type Ia.
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.
Kim
So Youn
SY
Section on Cellular Differentiation, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892-1830, USA.
Chen
Li-Yuan
LY
Yiu
Wai Han
WH
Weinstein
David A
DA
Chou
Janice Y
JY
D000818:Animals; D002118:Calcium / Q000032:analysis / Q000378:metabolism; D054426:Chemokine CXCL2; D002634:Chemotaxis, Leukocyte / Q000187:drug effects / Q000502:physiology; D016207:Cytokines / Q000097:blood*; D005953:Glycogen Storage Disease Type I / Q000209:etiology* / Q000378:metabolism* / Q000473:pathology; D051379:Mice; D018345:Mice, Knockout; D015846:Monokines / Q000494:pharmacology; D009240:N-Formylmethionine Leucyl-Phenylalanine / Q000494:pharmacology; D009504:Neutrophils / Q000378:metabolism*; D016897:Respiratory Burst / Q000502:physiology
D054426:Chemokine CXCL2; D016207:Cytokines; D015846:Monokines; D009240:N-Formylmethionine Leucyl-Phenylalanine; D002118:Calcium