{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T00:31:16Z","timestamp":1791937876709,"version":"4.3.4"},"reference-count":31,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2011,8,24]],"date-time":"2011-08-24T00:00:00Z","timestamp":1314144000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2012,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Glycogen storage disease type 1b (GSD 1b) is caused by mutations in the Glucose\u20106\u2010phosphate transporter and is characterized by impaired glucose homeostasis. In addition, GSD\u20101b is associated with chronic neutropenia resulting in recurrent infections and inflammatory bowel disease. It is unclear whether the neutropenia is solely due to enhanced apoptosis of mature neutrophils or whether aberrant neutrophil development may also contribute. Here we demonstrate that hematopoietic progenitors from GSD\u20101b patients are not impaired in their capacity to develop into mature neutrophils. However, optimal survival of neutrophil progenitors from GSD\u20101b patients requires high glucose levels (&gt; 200 mg dl<jats:sup>\u22121<\/jats:sup>), suggesting that even under normoglycemic conditions these cells are more prone to apoptosis. Furthermore, analysis of cytokine levels in peripheral blood suggests an inflammatory state with an inverse correlation between the level of inflammation and the number of neutrophils. Finally, in some patients, with low numbers of peripheral blood neutrophils, high numbers of neutrophils were observed in the intestine. Together, these results suggest that the neutropenia observed in GSD\u20101b patients is not caused by impaired maturation, but may be caused by both increased levels of apoptosis and egress of neutrophils from the blood to the inflamed tissues.<\/jats:p>","DOI":"10.1007\/s10545-011-9379-4","type":"journal-article","created":{"date-parts":[[2011,8,23]],"date-time":"2011-08-23T18:44:56Z","timestamp":1314125096000},"page":"287-300","source":"Crossref","is-referenced-by-count":20,"title":["Survival, but not maturation, is affected in neutrophil progenitors from GSD\u20101b patients"],"prefix":"10.1002","volume":"35","author":[{"given":"Gepke","family":"Visser","sequence":"first","affiliation":[{"name":"Department of Metabolic Diseases Wilhelmina Children's Hospital, University Medical Center Utrecht Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wilco","family":"de Jager","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Centre for Cellular and Molecular Immunology University Medical Center Utrecht Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liesbeth P.","family":"Verhagen","sequence":"additional","affiliation":[{"name":"Department of Cell Biology University Medical Center Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. Peter A.","family":"Smit","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases, Beatrix Children's Hospital University Medical Center Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frits A.","family":"Wijburg","sequence":"additional","affiliation":[{"name":"Division of Metabolic Diseases, Department of Pediatrics, Academic Medical Center (AMC) Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Berent J.","family":"Prakken","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Centre for Cellular and Molecular Immunology University Medical Center Utrecht Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul J.","family":"Coffer","sequence":"additional","affiliation":[{"name":"Department of Cell Biology University Medical Center Utrecht The Netherlands"},{"name":"Center of Molecular and Cellular Intervention, Wilhelmina Children's Hospital University Medical Center Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miranda","family":"Buitenhuis","sequence":"additional","affiliation":[{"name":"Department of Hematology, Erasmus MC, Rotterdam Dr. Molewaterplein 50, Faculty building Office Ee1330F GE Rotterdam 3015 The Netherlands"},{"name":"Erasmus MC Stem Cell Institute for Regenerative Medicine, Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,8,24]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-004-1405-1"},{"key":"e_1_2_6_3_1","first-page":"691","article-title":"Infectious and bleeding complications in patients with glycogenosis Ib","volume":"139","author":"Ambruso DR","year":"1985","journal-title":"Am J Dis 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