نتایج جستجو برای: chronic granulomatous disease cgd
تعداد نتایج: 1773487 فیلتر نتایج به سال:
The X-linked form of chronic granulomatous disease (XCGD) results from mutations in the gene encoding gp9lPhox, a 91-kD membrane glycoprotein that is the larger subunit of the respiratory burst oxidase cytochrome b. In this study, a new retroviral vector for expression of human gp91PhoN, MSCV-h91Neo. based on murine stem cell virus vectors, was evaluated using a human X-CGD myeloid cell line...
Failure of a superoxide generating system, the NADPH oxidase, present in neutrophils and other phagocytes gives rise to chronic granulomatous disease (CGD), a group of single-gene inherited disorders all characterized by an extreme susceptibility to pyogenic infection, with potentially fatal consequences. About 30% of CGD cases are caused by an autosomally inherited deficiency of a 47-Kd cytopl...
Aspergillus fumigatus is a model fungal pathogen and a common cause of infection in individuals with the primary immunodeficiency chronic granulomatous disease (CGD). Although primarily considered a deficiency of innate immunity, CGD is also linked to dysfunctional T cell reactivity. Both CD4(+) and CD8(+) T cells mediate vaccine-induced protection from experimental aspergillosis, but the molec...
Absence of a functional nicotinamide adenine dinucleotide phosphate (NADPH) oxidase predisposes chronic granulomatous disease (CGD) patients to infection, and also to unexplained, exaggerated inflammation. The impaired recognition and removal (efferocytosis) of apoptotic neutrophils by CGD macrophages may contribute to this effect. We hypothesized that peroxisome proliferator-activated receptor...
Immunodeficiency in chronic granulomatous disease (CGD) is well characterized. Less understood are exaggerated sterile inflammation and autoimmunity associated with CGD. Impaired recognition and clearance of apoptotic cells resulting in their disintegration may contribute to CGD inflammation. We hypothesized that priming of macrophages (Ms) with IFN-γ would enhance impaired engulfment of apopto...
Studies in Chronic Granulomatous Disease showed two breakthroughs during this past decade. First, the discovery of 7 Nox/Duox family proteins, Noxo1 and Noxa1 (homologues of gp91(phox), p47(phox) and p67(phox)) may clarify novel physiological mechanisms for superoxide regulation in various organs, such as the regulation of blood pressure, mucosal defense system in respiratory/digestive tract an...
Small-molecule screen identifies reactive oxygen species as key regulators of neutrophil chemotaxis.
Neutrophil chemotaxis plays an essential role in innate immunity, but the underlying cellular mechanism is still not fully characterized. Here, using a small-molecule functional screening, we identified NADPH oxidase-dependent reactive oxygen species as key regulators of neutrophil chemotactic migration. Neutrophils with pharmacologically inhibited oxidase, or isolated from chronic granulomatou...
Neutrophils play a crucial role in host defense against microbial infections. During phagocytosis of invading bacteria or fungi, the phagocyte NADPH oxidase produces superoxide. The importance of the oxidase is exemplified by the genetic disorder known as chronic granulomatous disease (CGD). The neutrophils of CGD patients cannot produce superoxide, with the result that affected infants and chi...
نمودار تعداد نتایج جستجو در هر سال
با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید