Inhibitor Of Kappa Light Polypeptide Gene Enhancer In B Cells
Inhibitor Of Nuclear Factor Kappa B Kinase Alpha Subunit
Inhibitor of nuclear factor kappa-B kinase subunit alpha
Nuclear Factor Kappa B Inhibitor Kinase Alpha
Nuclear factor NF kappa B inhibitor kinase alpha
Nuclear factor NF-kappa-B inhibitor kinase alpha
Nuclear factor NFkappaB inhibitor kinase alpha
Nuclear Factor Of Kappa Light Chain Gene Enhancer In B Cells Inhibitor
Transcription factor 16
Acts as part of the IKK complex in the conventional pathway of NF-kappa-B activation and phosphorylates inhibitors of NF-kappa-B thus leading to the dissociation of the inhibitor/NF-kappa-B complex and ultimately the degradation of the inhibitor. As part of the non-canonical pathway of NF-kappa-B activation, the MAP3K14-activated CHUK/IKKA homodimer phosphorylates NFKB2/p100 associated with RelB, inducing its proteolytic processing to NFKB2/p52 and the formation of NF-kappa-B RelB-p52 complexes. Also phosphorylates NCOA3. Phosphorylates 'Ser-10' of histone H3 at NF-kappa-B-regulated promoters during inflammatory responses triggered by cytokines.
Involvement in disease
Defects in CHUK are the cause of cocoon syndrome (COCOS) [MIM:613630]; also known as fetal encasement syndrome. COCOS is a lethal syndrome characterized by multiple fetal malformations including defective face and seemingly absent limbs, which are bound to the trunk and encased under the skin.
Belongs to the protein kinase superfamily. Ser/Thr protein kinase family. I-kappa-B kinase subfamily. Contains 1 protein kinase domain.
Phosphorylated by MAP3K14/NIK, AKT and to a lesser extent by MEKK1, and dephosphorylated by PP2A. Autophosphorylated. Acetylation of Thr-179 by Yersinia yopJ prevents phosphorylation and activation, thus blocking the I-kappa-B signaling pathway.
Cytoplasm. Nucleus. Shuttles between the cytoplasm and the nucleus.