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

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

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Classifications


Resource Type: Bibliography, Diagram, Illustration, Journal article/Issue, Review
Audience Level: Undergraduate upper division 15-16, Graduate, Professional (degree program)

Author and Copyright


Authors and Editors: Gilles Courtois of Unit頤e Biologie Mol飵laire de l'Expression G鮩que, Institut Pasteur, Alain Isra묍 of Unit頤e Biologie Mol飵laire de l'Expression G鮩que, Institut Pasteur
Publisher: American Association for the Advancement of Science
Format: application/pdf, image/gif, image/jpeg, text/html
Copyright and other restrictions: Yes
Cost: Yes

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