Regulatory subunit of the IKK core complex which 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. Its binding to scaffolding polyubiquitin seems to play a role in IKK activation by multiple signaling receptor pathways. However, the specific type of polyubiquitin recognized upon cell stimulation (either 'Lys-63'-linked or linear polyubiquitin) and its functional importance is reported conflictingly. Also considered to be a mediator for TAX activation of NF-kappa-B. Could be implicated in NF-kappa-B-mediated protection from cytokine toxicity. Essential for viral activation of IRF3. Involved in TLR3- and IFIH1-mediated antiviral innate response; this function requires 'Lys-27'-linked polyubiquitination.Xiao G., Oncogene 19:5198-5203(2000).Hong X., FEBS Lett. 499:133-136(2001).Wu R.-C., Mol. Cell. Biol. 22:3549-3561(2002).
Host
Rabbit
Immunogen
Synthesized peptide derived from internal of Human IKK-γ.
Involvement In Disease
Ectodermal dysplasia, anhidrotic, with immunodeficiency X-linked (EDAID); Ectodermal dysplasia, anhidrotic, with immunodeficiency, osteopetrosis and lymphedema (OLEDAID); Immunodeficiency, NEMO-related, without anhidrotic ectodermal dysplasia (NEMOID); Immunodeficiency 33 (IMD33); Recurrent isolated invasive pneumococcal disease 2 (IPD2); Incontinentia pigmenti (IP)
Raised In
Rabbit
Reactivity
Human
Regulatory
RUO
Relevance
Regulatory subunit of the IKK core complex which 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. Its binding to scaffolding polyubiquitin seems to play a role in IKK activation by multiple signaling receptor pathways. However, the specific type of polyubiquitin recognized upon cell stimulation (either 'Lys-63'-linked or linear polyubiquitin) and its functional importance is reported conflictingly. Also considered to be a mediator for TAX activation of NF-kappa-B. Could be implicated in NF-kappa-B-mediated protection from cytokine toxicity. Essential for viral activation of IRF3. Involved in TLR3- and IFIH1-mediated antiviral innate response; this function requires 'Lys-27'-linked polyubiquitination.
Xiao G., Oncogene 19:5198-5203(2000). Hong X., FEBS Lett. 499:133-136(2001). Wu R.-C., Mol. Cell. Biol. 22:3549-3561(2002).
Species
Homo Sapiens (Human)
Specificity
The antibody detects endogenous levels of total IKK-γ protein.
Regulatory subunit of the IKK core complex which 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. Its binding to scaffolding polyubiquitin seems to play a role in IKK activation by multiple signaling receptor pathways. However, the specific type of polyubiquitin recognized upon cell stimulation (either 'Lys-63'-linked or linear polyubiquitin) and its functional importance is reported conflictingly. Also considered to be a mediator for TAX activation of NF-kappa-B. Could be implicated in NF-kappa-B-mediated protection from cytokine toxicity. Essential for viral activation of IRF3. Involved in TLR3- and IFIH1-mediated antiviral innate response; this function requires 'Lys-27'-linked polyubiquitination.