PHAGOCYTES, GRANULOCYTES, AND MYELOPOIESIS A new genetic subgroup of chronic granulomatous disease with autosomal recessive mutations in p40phox and selective defects in neutrophil NADPH oxidase activity
نویسندگان
چکیده
1Wells Center for Pediatric Research, Department of Pediatrics, Indiana University School of Medicine, Indianapolis; 2Department of Pediatrics, Alberta Children’s Hospital, University of Calgary, Calgary, AB; 3Department of Pediatrics and Program in Immunology, Stanford University School of Medicine, CA; 4Departments of Microbiology and Infectious Disease, and Community of Health Sciences, and 5Department of Pathology, Alberta Children’s Hospital, University of Calgary, Calgary, AB; 6Departments of Biochemistry and Molecular Biology, and Center for Computational Biology and Bioinformatics, Indiana University School of Medicine, Indianapolis; 7Inflammation Program, Department of Medicine, Veterans Administration Medical Center Iowa City, and Roy J. and Lucille A. Carver College of Medicine, University of Iowa; and 8Departments of Microbiology/Immunology, and Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis
منابع مشابه
PHAGOCYTES, GRANULOCYTES, AND MYELOPOIESIS Lack of glucose recycling between endoplasmic reticulum and cytoplasm underlies cellular dysfunction in glucose-6-phosphatase- –deficient neutrophils in a congenital neutropenia syndrome
G6PC3 deficiency, characterized by neutropenia and neutrophil dysfunction, is caused by deficiencies in the endoplasmic reticulum (ER) enzyme glucose-6-phosphatase(G6Paseor G6PC3) that converts glucose6-phosphate (G6P) into glucose, the primary energy source of neutrophils. Enhanced neutrophil ER stress and apoptosis underlie neutropenia in G6PC3 deficiency, but the exact functional role of G6P...
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Alternatively activated macrophages, generated in a T-helper 2 environment, have demonstrated roles in wound repair and tissue remodeling in addition to being charged with immune tasks. Because the hydrolytic chemistries of the phagosomal lumen are central to many of these functions, we investigated their modification after alternative activation with IL-4 and IL-13. Most significantly, we foun...
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1Department of Pathology, Harvard Medical School, Dana-Farber/Harvard Cancer Center, and Department of Laboratory Medicine, Children’s Hospital Boston, MA; 2Department of Immunology, Children’s Hospital Boston, and Harvard Medical School, MA; 3Molecular and Integrative Physiological Sciences, Harvard School of Public Health, Boston, MA; and 4Department of Pathology and Laboratory Medicine, Emor...
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During hematopoiesis, myeloid cell leukemia-1 (MCL-1) mediates the survival of bone marrow progenitors and lymphocytes. However, its requirement during myeloid cell differentiation, development, and effector function is less clear. Lineage-specific deletion of MCL-1 in myeloid precursors results in neutropenia due to death during differentiation. The loss of mature neutrophils induced by Mcl-1 ...
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WHIM syndrome is a condition in which affected persons have chronic peripheral neutropenia, lymphopenia, abnormal susceptibility to human papilloma virus infection, and myelokathexis. Myelokathexis refers to the retention of mature neutrophils in the bone marrow (BM), which accounts for degenerative changes and hypersegmentation. Most patients present heterozygous autosomal dominant mutations o...
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