Article
The same IkappaBalpha mutation in two related individuals leads to completely different clinical syndromes.
The Journal of experimental medicine - 6 Sept 2004
Janssen Riny, van Wengen Annelies, Hoeve Marieke A, ten Dam Monique, van der Burg Miriam, van Dongen Jacques, van de Vosse Esther, van Tol Maarten, Bredius Robbert, Ottenhoff Tom H, Weemaes Corry, van Dissel Jaap T, Lankester Arjan
Abstract excerpt
Both innate and adaptive immune responses are dependent on activation of nuclear factor kappaB (NF-kappaB), induced upon binding of pathogen-associated molecular patterns to Toll-like receptors (TLRs). In murine models, defects in NF-kappaB pathway are often lethal and viable knockout mice have severe immune defects. Similarly, defects in the human NF-kappaB pathway described to date lead to severe clinical...
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