Bacterial handling defect in macrophages of patients with defects in glucose-6-phosphate metabolism and Crohn's disease like intestinal inflammation
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- Pandey, S; Balagopal, K; Aschenbrenner, D; Capitani, M; Weidinger, C; Pires, E; McCullagh, J; Travis, S; Ziegler, J; Siegmund, B; Uhlig, H
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Aberrant innate bacterial handling is one of the key mechanisms underlining Crohn’s disease (CD). Recently, in several inflammatory disorders defects in immunometabolism have been described but it is not clear whether this is a primary or secondary effect. Several monogenic immunodeficiencies that affect phagocyte activity present as Mendelian disorder associated inflammatory bowel disease. One intriguing subgroup is the group of patients with Glycogen storage disease type Ib (GSD-Ib) and congenital neutropenia. These patients have defective Glucose-6-phosphate metabolism caused due to genetic mutation in Glucose-6-phosphatase b and Glucose-6 phosphate-translocase (G6PT) and a subgroup develops CD-like intestinal inflammation.