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2018 ; 9
(ä): 613
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Nuclear Factor-kappaB in Autoimmunity: Man and Mouse
#MMPMID29686669
Miraghazadeh B
; Cook MC
Front Immunol
2018[]; 9
(ä): 613
PMID29686669
show ga
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.