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Production of oxygen radicals by fibroblasts and neutrophils from a patient with x-linked chronic granulomatous disease


European Journal of Haematology 51 (1993), pp.223-227
ISSN: 0902-4441
Journal Article
Fraunhofer ITA ( ITEM) ()
chronic granulomatous disease; fibroblast; immune system; immunology; neutrophils; phagocyte; prenatal diagnosis; reactive oxygen intermediate

Recently, a superoxide-generating NADPH-oxidase system in human fibroblasts has been described. Therefore, we reassessed the possible use of this cell type for prenatal diagnosis of CGD patients comparing normal and CGD peripheral blood neutrophils (PMN) and skin fibroblasts in their reactive oxygen intermediate (ROI)-producing capacity. While PMN of the CGD patient showed a clearly reduced respiratory burst activity, which correlated well with the measured content of cytochrome b558, fibroblasts of the same individual showed no impaired production of superoxide aneon or H2O2 upon stimulation by cytokines (TNF and IL-1) or other agents. Furthermore, fibroblasts of the CGD patient or of normal donors could be inhibited in ROI production by diphenylene iodonium (DPI) and 2-iodobiphenyl. In contrast to PMN, no inhibition of the fibroblast NADPH-oxidases system was observed using staurosporin an inhibitor of proteinkinase C. (Abstract truncated)