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Details on Person I kappa B kinase B (IKBKB or IKK beta) is the catalytic subu...
| Class:Id | Summation:5228867 |
|---|---|
| _displayName | I kappa B kinase B (IKBKB or IKK beta) is the catalytic subu... |
| _timestamp | 2014-10-06 04:32:27 |
| created | [InstanceEdit:5228861] Shamovsky, V, 2014-01-14 |
| literatureReference | [LiteratureReference:5228878] Deficiency of innate and acquired immunity caused by an IKBKB mutation |
| modified | [InstanceEdit:5602312] Shamovsky, Veronica, 2014-06-24 [InstanceEdit:5609671] Shamovsky, Veronica, 2014-07-16 [InstanceEdit:5625554] Shamovsky, Veronica, 2014-10-06 |
| text | I kappa B kinase B (IKBKB or IKK beta) is the catalytic subunit of the IKK complex, which also contains kinase IKK1 (IKBKA/IKKalpha) and a regulatory subunit NEMO (IKBKG/IKKgamma). The IKK complex phosphorylates inhibitors of NFkB (IkB) in response to multiple stimuli. The phosphorylation of the IkB proteins triggers their polyubiquitination and subsequent degradation by the 26S proteasome. Free NFkB dimer translocates to the nucleus where it regulates the expression of target genes. Defects in NFkB signaling can affect both innate and adaptive immune-receptor pathways. Homozygous duplication c.1292dupG in exon 13 of the IKBKB gene results in loss of expression of IKBKB (IKK beta). This mutation was identified in several patients diagnosed with severe combined immunodeficiency (SCID), a rare, potentially fatal, inherited disorderss associated with lack of mature B- and T-lymphocytes (Pannicke U et al. 2013). Patient-derived fibroblasts showed impaired NFkB-mediated responses upon stimulation with toll like receptor (TLR) ligands such as flagellin and lipopolysaccharide (LPS). Degradation of IkB alpha was absent upon TLR5 stimulation by flagellin. In addition, IL6 production was reduced in response to flagellin or lipopolysaccharide (which is specifically recognized by TLR4) (Pannicke U et al. 2013). Patient-derived B cells produced no immunoglobulins upon stimulation with TLR9 antagonist CpG (Pannicke U et al. 2013). |
| (summation) | [FailedReaction:5228840] Defective IKBKB (IKK2) does not form a complex with IKBKA and IKBKG [Homo sapiens] |
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