NF-κB signaling through its NFKB1-dependent canonical and NFKB2-dependent
noncanonical pathways plays distinctive roles in a diverse range of immune processes …

Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by
antibody deficiency, poor humoral response to antigens, and recurrent infections. To …

Non-canonical NF-κB-pathway signaling is integral in immunoregulation. Heterozygous
mutations in NFKB2 have recently been established as a molecular cause of common …

Background Genetic faults in several components of the nuclear factor-κB pathway cause
immunodeficiency. Most defects lead to combined immunodeficiency with a range of …

Autosomal dominant (AD) NFKB1 deficiency is thought to be the most common genetic
etiology of common variable immunodeficiency (CVID). However, the causal link between …

Background The nuclear factor κ light-chain enhancer of activated B cells (NF-κB) signaling
pathway is a key regulator of immune responses. Accordingly, mutations in several NF-κB …

In common variable immunodeficiency (CVID), heterozygous damaging NFKB1 variants
represent the most frequent monogenic cause. NFKB1 encodes the precursor p105, which …

Nuclear factor κ light-chain enhancer of activated B cells (NF-κB) family of evolutionarily
conserved transcription factors are involved in key cellular signaling pathways. Previously …

Mutations affecting the non-canonical pathway of NF-κB were recently identified to underlie
a form of common variable immunodeficiency strongly associated with autoimmunity …

Most of the currently known heterozygous pathogenic NFKB1 (Nuclear factor kappa B
subunit 1) variants comprise deleterious defects such as severe truncations, internal …