How do nuclear factor kappa B (NF-kappa B)1 and NF-kappa B2 defects lead to the incidence of clinical and immunological manifestations of inborn errors of immunity?

Expert review of clinical immunology(2023)

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摘要
Introduction: Genetic defects affect the manner of the immune system's development, activation, and function. Nuclear factor-kappa B subunit 1 (NF-kappa B1) and NF-kappa B2 are involved in different biological processes, and deficiency in these transcription factors may reveal clinical and immunological difficulties.Areas covered: This review article gathers the most frequent clinical and immunological remarkable characteristics of NF-kappa B1 and NF-kappa B2 deficiencies. Afterward, an effort is made to describe the biological mechanism, which is likely to be the cause of these clinical and immunological abnormalities.Expert opinion: The present review article has explained the mechanism of contributions of the NF-kappa B1 and NF-kappa B2 deficiency in revealing immunodeficiency symptoms, specifically immunological and clinical manifestations. These mechanisms demonstrate the importance of NF-kappa B1 and NF-kappa B2 signaling path-ways for B and T cell development, activation, antibody production, and immunotolerance. The manifestation of a mutation can range from no symptoms to severe complications in a family.
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关键词
Antibody deficiency,common variable immunodeficiency,inborn errors of immunity,NF-kappa B1,NF-kappa B2,nuclear factor kappa B,primary immunodeficiency
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