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Defective lymphoid organogenesis underlies the immune deficiency caused by a heterozygous S32I mutation in IκBα.
Jana L Mooster, Severine Le Bras, Michel J Massaad, Haifa Jabara, Juhan Yoon, Claire Galand, Balthasar A Heesters, Oliver T Burton, Hamid Mattoo, John Manis, Raif S Geha
Jun 27, 2019
Patients with ectodermal dysplasia with immunodeficiency (ED-ID) caused by mutations in the inhibitor of NF-κB α (IκBα) are susceptible to severe recurrent infections, despite normal T and B cell numbers and intact in vitro...
Animals Antibody Formation B-Lymphocytes Codon Dermatitis Contact Disease Models Animal Heterozygote Hypersensitivity Delayed I-kappa B Proteins Immunologic Deficiency Syndromes Lymphoid Tissue Mice Mice Transgenic Mutation NF-KappaB Inhibitor alpha Organogenesis Phosphorylation Proteolysis T-Lymphocyte Subsets Toll-Like Receptors Tumor Necrosis Factors
NF-κB activation is a turn on for vaccinia virus phosphoprotein A49 to turn off NF-κB activation.
innate immunity nuclear factor kappa B vaccinia virus virus immune evasion Feedback Physiological Humans I-kappa B Kinase I-kappa B Proteins Molecular Mimicry NF-kappa B Phosphoproteins Phosphorylation Signal Transduction Ubiquitin-Protein Ligases Ubiquitination vaccinia virus Viral Proteins Virulence beta-Transducin Repeat-Containing Proteins