Work

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

Year: 2007

Type: article

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.

Source: FEBS Letters

DOI: 10.1016/j.febslet.2007.07.013

Authors: So Youn Kim, Li-Yuan Chen, Wai Han Yiu, David A. Weinstein, Janice Y. Chou

Institutions: National Institutes of Health, Eunice Kennedy Shriver National Institute of Child Health and Human Development, University of Florida Health, University of Florida

Language: English

Topic: Glycogen Storage Diseases and Myoclonus

Subfield: Rheumatology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: green

Funders: National Institutes of Health, Eunice Kennedy Shriver National Institute of Child Health and Human Development