Anti-Human IKBKG Antibody Pair

Product Description: Inhibitor of nuclear factor kappa B kinase regulatory subunit gamma (IKBKG), also known as NEMO (NF-kappa B essential modulator), is the regulatory subunit of the inhibitor of nuclear factor kappa-B kinase (IKK) complex, critical for activating the NF-kappaB signaling pathway. Structurally, NEMO serves as a scaffold that assembles and activates the IKK complex by bridging catalytic subunits IKKalpha and IKKbeta, leading to phosphorylation and degradation of IkappaB inhibitors and release of NF-kappaB transcription factors. This activation controls genes regulating inflammation, immunity, cell survival, and development. Beyond its role in NF-kappaB signaling, NEMO also promotes autophagosomal clearance of misfolded protein aggregates, contributing to proteostasis. Pathogenic mutations in IKBKG cause incontinentia pigmenti (IP), an X-linked dominant disorder causing skin, hair, teeth, and central nervous system abnormalities, primarily affecting females due to male lethality. Mutations also underlie ectodermal dysplasia with immunodeficiency and progressive neurodegeneration resulting from defective NF-kappaB signaling and impaired protein quality control. Protein Function: 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 (PubMed:14695475, PubMed:20724660, PubMed:21518757, PubMed:9751060). Its binding to scaffolding polyubiquitin plays a key role in IKK activation by multiple signaling receptor pathways (PubMed:16547522, PubMed:18287044, PubMed:19033441, PubMed:19185524, PubMed:21606507, PubMed:27777308, PubMed:33567255). Can recognize and bind both 'Lys-63'-linked and linear polyubiquitin upon cell stimulation, with a much higher affinity for linear polyubiquitin (PubMed:16547522, PubMed:18287044, PubMed:19033441, PubMed:19185524, PubMed:21606507, PubMed:27777308). Could be implicated in NF-kappa-B-mediated protection from cytokine toxicity. Essential for viral activation of IRF3 (PubMed:19854139). Involved in TLR3- and IFIH1-mediated antiviral innate response, this function requires 'Lys-27'-linked polyubiquitination (PubMed:20724660) , (Microbial infection) Also considered to be a mediator for HTLV-1 Tax oncoprotein activation of NF-kappa-B [The Uniprot Consortium]