Sci. STKE, 14 November 2000
NF-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-B (NF-B) family of transcription factors are critical for regulating the response to immune challenges. Recently, a role for NF-B in skin biology has been revealed. Within the cascade of proteins whose activities impinge upon the activation of NF-B, the NEMO (NF-B essential modulator)/IKK protein is required for the activation of the IB kinases, which in turn, promote the degradation of IB proteins, leading to the derepression of NF-B activity. Courtois and Israël discuss the role of NEMO/IKK in normal physiological activation of NF-B and the consequences of defective NF-B activation, as an effect of NEMO/IKK 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-B Defects in Humans: The NEMO/Incontinentia Pigmenti Connection. Sci. STKE 2000, pe1 (2000).
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