NF-?B pathway and the Goldilocks principle: Lessons from human disorders of immunity and inflammation - 04/05/19
Abstract |
Nuclear factor κ-light-chain-enhancer of activated B cells (NF-κB) signaling pathways play a key role in various cell processes related to host immunity. The last few years have seen an explosion of disorders associated with NF-κB components from core members of the canonical and noncanonical cascades to adaptor protein and ubiquitination-related enzymes. Disease phenotypes have extended beyond susceptibility to infections and include autoimmunity, lymphoproliferation, atopy, and inflammation. Concurrently, studies are unveiling a tightly regulated system marked by extensive cross-talk between the canonical and noncanonical pathways, as well as among the NF-κB and other signaling pathways. As the rate of discovery in the realm of NF-κB defects accelerates, this review presents a timely summary of major known defects causing human disease, as well as diagnostic, therapeutic, and research challenges and opportunities.
Le texte complet de cet article est disponible en PDF.Key words : NF-κB, canonical, noncanonical, signaling, immune regulation
Abbreviations used : BCL10, CARD, CVID, GOF, HOIL-1, HOIP, HSCT, IKBKB, IKK, IL-1R, IP, IRAK, LOF, LTβR, LUBAC, MALT1, MyD88, NEMO, NF-κB, NFKBIA, NIK, NK, OTULIN, SCID, TCR, TLR, TRAF, Treg, XL-EDA-ID
Plan
Disclosure of potential conflict of interest: The authors declare that they have no relevant conflicts of interest. |
Vol 143 - N° 5
P. 1688-1701 - mai 2019 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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