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Nuclear Factor-kappaB in Autoimmunity: Man and Mouse.


ABSTRACT: NF-?B (nuclear factor-kappa B) is a transcription complex crucial for host defense mediated by innate and adaptive immunity, where canonical NF-?B signaling, mediated by nuclear translocation of RelA, c-Rel, and p50, is important for immune cell activation, differentiation, and survival. Non-canonical signaling mediated by nuclear translocation of p52 and RelB contributes to lymphocyte maturation and survival and is also crucial for lymphoid organogenesis. We outline NF-?B signaling and regulation, then summarize important molecular contributions of NF-?B to mechanisms of self-tolerance. We relate these mechanisms to autoimmune phenotypes described in what is now a substantial catalog of immune defects conferred by mutations in NF-?B pathways in mouse models. Finally, we describe Mendelian autoimmune syndromes arising from human NF-?B mutations, and speculate on implications for understanding sporadic autoimmune disease.

SUBMITTER: Miraghazadeh B 

PROVIDER: S-EPMC5900062 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

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Nuclear Factor-kappaB in Autoimmunity: Man and Mouse.

Miraghazadeh Bahar B   Cook Matthew C MC  

Frontiers in immunology 20180409


NF-κB (nuclear factor-kappa B) is a transcription complex crucial for host defense mediated by innate and adaptive immunity, where canonical NF-κB signaling, mediated by nuclear translocation of RelA, c-Rel, and p50, is important for immune cell activation, differentiation, and survival. Non-canonical signaling mediated by nuclear translocation of p52 and RelB contributes to lymphocyte maturation and survival and is also crucial for lymphoid organogenesis. We outline NF-κB signaling and regulati  ...[more]

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