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Sci. STKE, 14 November 2000
Vol. 2000, Issue 58, p. pe1
[DOI: 10.1126/stke.2000.58.pe1]


NF-{kappa}B Defects in Humans: The NEMO/Incontinentia Pigmenti Connection

Gilles Courtois and Alain Israël

The authors are in the Unité de Biologie Moléculaire de l'Expression Génique, URA 1773 CNRS, Institut Pasteur 75724 Paris Cedex 15, France.

Abstract: The components of the nuclear factor-{kappa}B (NF-{kappa}B) family of transcription factors are critical for regulating the response to immune challenges. Recently, a role for NF-{kappa}B in skin biology has been revealed. Within the cascade of proteins whose activities impinge upon the activation of NF-{kappa}B, the NEMO (NF-{kappa}B essential modulator)/IKK{gamma} protein is required for the activation of the I{kappa}B kinases, which in turn, promote the degradation of I{kappa}B proteins, leading to the derepression of NF-{kappa}B activity. Courtois and Israël discuss the role of NEMO/IKK{gamma} in normal physiological activation of NF-{kappa}B and the consequences of defective NF-{kappa}B activation, as an effect of NEMO/IKK{gamma} mutations, which can lead to incontinentia pigmenti, a disease marked by alopecia, tooth eruption, skin lesions, and changes in skin pigmentation.

Citation: G. Courtois, A. Israël, NF-{kappa}B Defects in Humans: The NEMO/Incontinentia Pigmenti Connection. Sci. STKE 2000, pe1 (2000).

Read the Full Text

NF-{kappa}B Essential Modulator (NEMO) Interaction with Linear and Lys-63 Ubiquitin Chains Contributes to NF-{kappa}B Activation.
K. Hadian, R. A. Griesbach, S. Dornauer, T. M. Wanger, D. Nagel, M. Metlitzky, W. Beisker, M. Schmidt-Supprian, and D. Krappmann (2011)
J. Biol. Chem. 286, 26107-26117
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