How Do Nuclear Factor Kappa B (Nf-Κb)1 and Nf-Κb2 Defects Lead to the Incidence of Clinical and Immunological Manifestations of Inborn Errors of Immunity? Publisher Pubmed



Fathi N^{1, 2} ; Mojtahedi H^{2, 3} ; Nasiri M¹ ; Abolhassani H^{1, 4} ; Yousefpour Marzbali M^{1, 5} ; Esmaeili M^{1, 2} ; Salami F^{1, 2} ; Biglari F⁶ ; Rezaei N^{1, 2, 7}
Source: Expert Review of Clinical Immunology Published:2023

Abstract

Introduction: Genetic defects affect the manner of the immune system’s development, activation, and function. Nuclear factor-kappa B subunit 1 (NF-κB1) and NF-κB2 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-κB1 and NF-κB2 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-κB1 and NF-κB2 deficiency in revealing immunodeficiency symptoms, specifically immunological and clinical manifestations. These mechanisms demonstrate the importance of NF-κB1 and NF-κB2 signaling pathways 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. © 2023 Informa UK Limited, trading as Taylor & Francis Group.