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Molecular and Cellular Pediatrics
Fig. 4 | Molecular and Cellular Pediatrics

Fig. 4

From: Autoimmune lymphoproliferative immunodeficiencies (ALPID) in childhood: breakdown of immune homeostasis and immune dysregulation

Fig. 4

Illustration of the NF-κB pathway. The NF-κB1 transcriptional factor (p50 and its precursor p105) is active upon dimerization (p50:p65) in the cytosol. At rest, NF-κB dimers are bound to inhibitory IκB proteins. After activation (here via PRR signaling), IκB proteins are phosphorylated by the IκB kinase (IKK) complex, which releases the NF-κB dimers. p65-containing heterodimers can then translocate into the nucleus and regulate gene expression. Heterozygous loss-of-function mutations in NFKB1 are associated with reduced protein levels of the p105 and/or p50 subunit and lead to the development of a complex immunodeficiency

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