Neutrophilia and elevated serum cytokines are implicated in glycogen storage disease type Ia.
FEBS Lett
; 581(20): 3833-8, 2007 Aug 07.
Article
in En
| MEDLINE
| ID: mdl-17659284
ABSTRACT
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Glycogen Storage Disease Type I
/
Cytokines
/
Neutrophils
Limits:
Animals
Language:
En
Journal:
FEBS Lett
Year:
2007
Type:
Article
Affiliation country:
United States