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- W2058907025 abstract "The production of hydrogen peroxide in phagccytosing leukocytes is essential for the killing of microorganisms. Glutathione is thought to be involved in two ways in this process: 1) in the detoxification of the excess of H2O2; 2) as hydrogen donor in the production of H2O2. We were able to study the actual role of the glutathione redox system in the first known family with a real glutathione reductase deficiency in all blood cells. We found that in contrast to normal cells, the oxygen consumption and H2O2 generation abruptly stopped 5-10 minutes after the onset of phagocytosis. This effect was probably due to cell injury since the patients' granulocytes and monocytes, after pre-incubation with an H2O2-generating system, did not react any longer with phagocytosable particles. The generation of superoxide radicals (O−2), intermediates in the formation of H2O2, was normal in the patients' granulocytes, indicating that glutothione is not involved in the production of these compounds. Most likely, the cytochrome c, used for the detection of O−2, prevented efficient generation of H2O2, thus protecting the cells against its harmful effects. The lack of recurrent infections in these patients and the normal in vitro phagocytosis and killing of Stophylococcus aureus indicate that the short respiratory burst suffices for effective host defense. Moreover, the occurrence of hemolytic crises after eating of fava beans and the development of cataracts in this family indicate that glutathione fulfils an essential role in the protection of many different cells against oxidative stress." @default.
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- W2058907025 date "1978-01-01" @default.
- W2058907025 modified "2023-10-17" @default.
- W2058907025 title "ROLE OF GLUTATHIONE IN PHAGOCYTE HOST DEFENSE MECHANISMS" @default.
- W2058907025 doi "https://doi.org/10.1203/00006450-197801000-00077" @default.
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