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.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 51 print issues and online access
$199.00 per year
only $3.90 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
Drachman, D. B. New Engl. Med J. 298, 136–142 (1978).
Rose, N. R., Lorenzi, M. & Lewis, M. in Basic and Clinical Immunology (eds Stites, D. P. et al.) 652–655 (Lange, California, 1984).
Petz, L. D. & Garratty, G. in Acquired Immune Haemolytic Anaemia (Churchill Livingstone, London, 1980).
Lancet i, 255 (1981).
Lachmann, P. J. & Peters, D. K. in Clinical Aspects of Immunology (eds Lachmann, P. J. & Peters, D. K.) 38–40 (Blackwell, Oxford, 1982).
Kaplan, A. P. et al. CRC Crit. Rev. Immun. 75–93 (1981).
Sim, R. B. & Reboul, A. Meth. Enzym. 80, 43–54 (1981).
Bloom, A. L. & Thomas, A. P. in Haemostasis and Thrombosis (Churchill Livingstone, London, 1981).
Sheffer, A. L. et al. J. Allerg. clin. Immun. 75, 640–646 (1985).
Haines, A. L. & Lepow, I. H. J. Immun. 92, 456–467 (1963).
Fahey, J. L. & Terry, E. W. in Handbook of Experimental Immunology (ed. Weir, D. M.) 8.3–8.10 (Blackwell, Oxford, 1979).
Frank, M. M., Gelfand, J. A. & Atkinson, J. P. Ann. intern. Med. 84, 580–593 (1976).
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
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
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1038/323722a0
This article is cited by
-
C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency
Orphanet Journal of Rare Diseases (2023)
-
The Immunopathology of Complement Proteins and Innate Immunity in Autoimmune Disease
Clinical Reviews in Allergy & Immunology (2020)
-
Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate
Orphanet Journal of Rare Diseases (2019)
-
Acquired Angioedema: A Rare Manifestation of Angioimmunoblastic T Cell Lymphoma
Indian Journal of Otolaryngology and Head & Neck Surgery (2019)
-
Cellular proliferation in mouse and human pancreatic islets is regulated by serpin B13 inhibition and downstream targeting of E-cadherin by cathepsin L
Diabetologia (2019)
Comments
By submitting a comment you agree to abide by our Terms and Community Guidelines. If you find something abusive or that does not comply with our terms or guidelines please flag it as inappropriate.