Abstract
Phagocytosing neutrophils, monocytes, macrophages and eosino-phils produce a burst of non-mitochondrial respiration that is important for the killing and digestion of microbes. Much of the information about the oxidase system involved comes from studies on patients with chronic granulomatous disease (CGD), a syndrome in which an undue predisposition to infection results from complete absence of this burst of stimulated respiratory activity1. The basis of the oxidase activity is an electron transport chain, the only established component of which is a very unusual b-type cyto-chrome (b−245) (ref. 2). The molecular defect in the X-linked subgroup of CGD is the absence of this cytochrome b−245, which, however, appears to be normal in those subjects with the autosomal recessive mode of inheritance3. In an attempt to identify an abnormality of activation, or an absence or malfunction of a proximal component of the electron transport chain in this latter group, we examined protein phosphorylation in neutrophils after activation of the oxidase with phorbol myristate acetate. All four of the patients studied demonstrated a selective lack of the enhanced phosphorylation of a protein of relative molecular mass (Mr) 44,000 (44K) that was observed in normal subjects and in two CGD patients with an X-linked inheritance. This molecule, therefore, could be an important functional component of the oxidase.
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References
Holmes, B., Page, A. R. & Good, R. A. J. clin. Invest. 46, 1422–1432 (1967).
Segal, A. W. & Jones, O. T. G. Nature 276, 515–517 (1978).
Segal, A. W. et al. New Engl. J. Med. 308, 245–251 (1983).
Nishizuka, Y. Nature 308, 693–698 (1984).
Schneider, C., Zanetti, M. & Romeo, D. FEBS Lett. 127, 4–8 (1981).
Andrews, P. C. & Babior, B. M. Blood 61, 333–340 (1983).
Andrews, P. C. & Babior, B. M. Blood 64, 883–890 (1984).
Segal, A. W. & Jones, O. T. G. Biochem. J. 182, 181–188 (1979).
Segal, A. W., Dorling, J. & Coade, S. J Cell Biol. 85, 42–59 (1980).
Hitzig, W. H. & Seger, R. A. Hum. Genet. 64, 207–215 (1983).
Segal, A. W. & Jones, O. T. G. FEBS Lett. 110, 111–114 (1980).
Cagan, R. H. & Karnovsky, M. L. Nature 204, 255–257 (1964).
Gabig, T. G. & Babior, B. M. J. biol Chem. 254, 9070–9074 (1979).
Badwey, J. A. & Karnovsky, M. L. A. Rev. Biochem. 49, 695–726 (1980).
Light, D. R., Walsh, C., O'Callaghan, A. M., Goetzel, E. J. & Tauber, A. I. Biochemistry 20, 1468–1476 (1981).
Cross, A. R., Jones, O. T. G., Garcia, R. & Segal, A. W. Biochem. J. 208, 759–763 (1982).
Packard, B. Trends biochem. Sci. 10, 138 (1985).
Cockroft, S. Biochim. biophys. Acta 794, 37–46 (1984).
Laemmli, U. K. Nature 227, 680–685 (1970).
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Segal, A., Heyworth, P., Cockcroft, S. et al. Stimulated neutrophils from patients with autosomal recessive chronic granulomatous disease fail to phosphorylate a Mr-44,000 protein. Nature 316, 547–549 (1985). https://doi.org/10.1038/316547a0
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DOI: https://doi.org/10.1038/316547a0
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