An IgG autoantibody which inactivates C1¯-inhibitor (original) (raw)

Nature volume 323, pages 722–724 (1986)Cite this article

Abstract

Antibodies are considered to play a specific pathogenic role in certain disease states such as myasthenia gravis, Graves' disease and autoimmune haemolytic anaemia1–3. Autoantibodies which interfere with the function of enzyme cascade systems have also been described in diseases such as acquired haemophilia (antifactor VIII antibodies) and glomerulonephritis (C3 nephritic factor)4,5. The identification of these autoantibodies is crucial to an understanding of the aetiology of such diseases and is also of importance in revealing the inter-relationships of the immune system with other biological pathways. This is the first report of an immunoglobulin G (IgG) autoantibody reactive with C1¯-inhibitor (C1¯-Inh), a pivotal inhibitor of the inflammatory response which is known to inactivate proteins of the complement, kinin, fibrinolytic arid ‘contact phase’ systems6,7. This autoantibody was isolated from a patient with a novel variant of acquired angioedema and C1¯-Inh dysfunction. This finding highlights the involvement of the immune system in the pathogenesis of disorders characterized by the presence of dysfunctional inflammatory response proteins.

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Author notes

  1. R. B. Sim: MRC Immunochemistry Unit, Department of Biochemistry, University of Oxford, Oxford OX1 3QU, UK

Authors and Affiliations

  1. Department of Immunology, St James Hospital and Trinity College, Dublin, 8, Ireland
    J. Jackson, R. B. Sim, A. Whelan & C. Feighery

Authors

  1. J. Jackson
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  2. R. B. Sim
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  3. A. Whelan
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  4. C. Feighery
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Jackson, J., Sim, R., Whelan, A. et al. An IgG autoantibody which inactivates C1¯-inhibitor.Nature 323, 722–724 (1986). https://doi.org/10.1038/323722a0

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