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IKK Regulation and Human Genetics

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NF-kB in Health and Disease

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 349))

Abstract

The IKK kinase complex is the core element of the NF-κB cascade. It is essentially made of two kinases (IKKα and IKKβ) and a regulatory subunit, NEMO/IKKγ. Additional components may exist, transiently or permanently, but their characterization is still uncertain. In this review, we will focus on the NEMO molecule, and describe the results which have been obtained, and the hypotheses which have been proposed, to explain how NEMO controls the activation of the IKK complex. NEMO is one of the very few non-redundant components of the NF-κB cascade, and the localization of the gene that encodes it on the X chromosome suggests it is likely to be the target of mutations leading to pathologies: this is indeed the case, and we will also present the current status of our knowledge regarding NEMO-associated pathologies.

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Authors and Affiliations

  1. INSERM U781, Hôpital Necker-Enfants Malades, Paris, France

    Gilles Courtois

  2. Unite de Signalisation Moleculaire et Activation Cellulaire, URA 2582 CNRS, Institut Pasteur, 25 rue du Dr Roux, 75724, Paris Cedex 15, France

    Alain Israël

Authors
  1. Gilles Courtois
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  2. Alain Israël
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Correspondence to Alain Israël .

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Editors and Affiliations

  1. School of Medicine, Dept. Pharmacology, University of California, San Diego, Gilman Dr. 9500, La Jolla, 92093-0636, California, USA

    Michael Karin

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Courtois, G., Israël, A. (2010). IKK Regulation and Human Genetics. In: Karin, M. (eds) NF-kB in Health and Disease. Current Topics in Microbiology and Immunology, vol 349. Springer, Berlin, Heidelberg. https://doi.org/10.1007/82_2010_98

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