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J. Biol. Chem., Vol. 281, Issue 39, 28794-28801, September 29, 2006

This Article Full Text Full Text (PDF) All Versions of this Article: 281/39/28794    most recent M604964200v1 Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kim, S. Y. Articles by Chou, J. Y. Search for Related Content PubMed PubMed Citation Articles by Kim, S. Y. Articles by Chou, J. Y. Social Bookmarking                      What's this?

Bone Marrow-derived Cells Require a Functional Glucose 6-Phosphate Transporter for Normal Myeloid Functions^*

So Youn Kim, Andrew D. Nguyen, Ji-Liang Gao, Philip M. Murphy, Brian C. Mansfield^¶, and Janice Yang Chou¹

From the Section on Cellular Differentiation, Heritable Disorders Branch, NICHD, Molecular Signaling Section, Laboratory of Molecular Immunology, NIAID, National Institutes of Health, Bethesda, Maryland 20892 and ^¶Correlogic Systems, Inc., Rockville, Maryland 20850

Glycogen storage disease type Ib (GSD-Ib) is caused by a deficiency in the ubiquitously expressed glucose 6-phosphate transporter (Glc-6-PT). Glc-6-PT activity has been shown to be critical in the liver and kidney where a deficiency disrupts glucose homeostasis. GSD-Ib patients also have defects in the neutrophil respiratory burst, chemotaxis, and calcium flux. They also manifest neutropenia, but whether Glc-6-PT deficiency in the bone marrow underlies myeloid dysfunctions in GSD-Ib remains controversial. To address this, we transferred bone marrow from Glc-6-PT-deficient (Glc-6-PT^–/–) mice to wild-type mice to generate chimeric mice (BM-Glc-6-PT^–/–). As a control, we also transferred bone marrow between wild-type mice (BM-Glc-6-PT^+/+). While BM-Glc-6-PT^+/+ mice have normal myeloid functions, BM-Glc-6-PT^–/– mice manifest myeloid abnormalities characteristic of Glc-6-PT^–/– mice. Both have impairments in their neutrophil respiratory burst, chemotaxis response, and calcium flux activities and exhibit neutropenia. In the bone marrow of BM-Glc-6-PT^–/– and Glc-6-PT^–/– mice, the numbers of myeloid progenitor cells are increased, while in the serum there is an increase in granulocyte colony-stimulating factor and chemokine KC levels. Moreover, in an experimental model of peritoneal inflammation, local production of KC and the related chemokine macrophage inflammatory protein-2 is decreased in both BM-Glc-6-PT^–/– and Glc-6-PT^–/– mice along with depressed peritoneal neutrophil accumulation. The neutrophil recruitment defect was less severe in BM-Glc-6-PT^–/– mice than in Glc-6-PT^–/– mice. These findings demonstrate that Glc-6-PT expression in bone marrow and neutrophils is required for normal myeloid functions and that non-marrow Glc-6-PT activity also influences some myeloid functions.

Received for publication, May 23, 2006 , and in revised form, August 2, 2006.

^* This work was supported in part by the Intramural Research Program of the NICHD, National Institutes of Health. The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

¹ To whom correspondence should be addressed: Bldg. 10, Rm. 9D42, National Institutes of Health, Bethesda, MD 20892-1830. Tel.: 301-496-1094; Fax: 301-402-6035; E-mail: chouja{at}mail.nih.gov.

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