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The protective role of glutathione

The Effect of Congenital Defects of Glutathione Metabolism on the Function of Erythrocytes, Eye Lens Cells, and Phagocytic Leukocytes. A Review and Some Personal Observations

  • Chapter
Inborn Errors of Immunity and Phagocytosis

Abstract

Many biological oxidations proceed via formation of radicals and/or peroxides. The tripeptide γ-glutamylcysteinylglycine or reduced glutathione (GSH) is known to protect cells against oxidative damage. This function can be executed in three different ways:

  1. 1.

    GSH, like other thiols, can act as a radical acceptor1, thereby forming the disulphide GSSG (oxidized glutathione):

    $$GSH + R. \to GS.RH2GS. \to GSSG$$
  2. 2.

    Peroxides may be eliminated either by catalase (in case of hydrogen (peroxide) or by GSH in the glutathione peroxidase reaction2:

  3. 3.

    Oxidative reactions may also give rise to formation of disulphide bridges in proteins, often leading to unfolding and denaturation of these proteins. Glutathione is able to restore the function of these proteins by reduction of such disulphides, with formation of mixed disulphides as intermediates3,4:

    $$GSH + R - S - S - R' \rightleftharpoons G - S - S - R + R' - SHGSH + G - S - S - R \rightleftharpoons GSSG + R - SH$$

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  1. D. Roos
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  2. R. S. Weening
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  3. J. A. Loos
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Editors and Affiliations

  1. John F. Kennedy Institute, DK-2600, Glostrup, Denmark

    F. Güttler

  2. Department of Chemical Pathology, Institute of Child Health, WC1N 1EH, London

    J. W. T. Seakins

  3. Division of Perinatal Medicine, MRC Clinical Research Centre, Harrow, HA1 3UJ, Middlesex, London

    R. A. Harkness

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Roos, D., Weening, R.S., Loos, J.A. (1979). The protective role of glutathione. In: Güttler, F., Seakins, J.W.T., Harkness, R.A. (eds) Inborn Errors of Immunity and Phagocytosis. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-6197-8_18

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  • DOI: https://doi.org/10.1007/978-94-011-6197-8_18

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