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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 plays a key role in IKK activation by multiple signaling receptor pathways. Can recognize and bind both 'Lys-63'-linked and linear polyubiquitin upon cell stimulation, with a much higher affinity for linear polyubiquitin. 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.; (Microbial infection) Also considered to be a mediator for HTLV-1 Tax oncoprotein activation of NF-kappa-B.
Gene References into Functions
Computational analysis identified two miR-107 binding sites in the 3'UTR of IKBKG suggesting that IKBKG expression is regulated by miR-107. PMID: 30396951
Human IKKgamma does not interact with mammalian Atg8-family proteins. PMID: 29097655
Data suggest the angiopoietin-like 8 (ANGPTL8)/p62-IKKgamma axis as a negative feedback loop that regulates NF-kappaB activation, and extends the role of selective autophagy in fine-tuned inflammatory responses. PMID: 29255244
this study demonstrates immunodeficiency in two female patients with Incontinentia Pigmenti with heterozygous NEMO mutation diagnosed by lipopolysaccharide unresponsiveness PMID: 28702714
GSK-3beta is critically important for ordered NF-kappaB signalling through modulation of NEMO phosphorylation. PMID: 27929056
HOTAIR operates the action of IKKalpha, IKKbeta, IKKgamma in liver cancer stem cells PMID: 27367027
the present study found that loss of the NEMO-SHARPIN interaction impaired recruitment of truncated NEMO forms into punctuate structures that are transiently formed on cell stimulation and thus led to a defect in linear ubiquitination PMID: 28249776
NEMO was critically involved in the cGAS-STING pathway. PMID: 28939760
Results show that NEMO's expression is regulated by ASAP3 which it interacts directly with it reducing its poly-ubiquitinylation. PMID: 28502111
E+P treatment of breast cancer cells increased ER binding to the NEMO promoter, thereby increasing NEMO expression. PMID: 28515148
Hematopoietic stem cell transplantation can cure most clinical features of patients with a variety of IKBKG mutations. PMID: 28679735
Authors show that NEMO stabilizes HIFalpha via direct interaction and independently of NF-kappaB signaling in vitro. NEMO prolongs tumor cell survival via regulation of apoptosis and activation of epithelial-to-mesenchymal transition, facilitating tumor metastasis. PMID: 26500060
The results demonstrate the the first example of father-to-daughter transmission of IP in which a pathogenic mutation in IKBKG has been demonstrated PMID: 27037530
Molluscum contagiosum virus MC005 inhibited NF-kappaB proximal to the IkappaB kinase (IKK) complex, and unbiased affinity purification revealed that MC005 interacts with the IKK subunit NEMO (NF-kappaB essential modulator). PMID: 28490597
These data suggest that molluscum contagiosum virus MC159 competitively binds to NEMO to prevent cIAP1-induced NEMO polyubiquitination. PMID: 28515292
High IKBKG expression is associated with multiple myeloma. PMID: 27454822
Our findings shed light on the nature of the interaction between NEMO and poly-ubiquitin, suggesting that NEMO is differentially regulated by poly-ubiquitin chain length and that this regulation occurs via a modulation of the available equilibrium of conformational states, rather than gross structural change PMID: 27028374
FADD, as well as NEMO, is a substrate for LUBAC ubiquitin ligase (E3) complex, composed of the HOIP, HOIL-1L, and SHARPIN subunits. PMID: 28189684
Consistent with experimental evidence, the zinc ion is essential for mechanical stabilization of the functional, folded conformation of NEMO. PMID: 28035815
Herein, our simulations of the zinc finger NEMO (2JVX) using multiple simulations of length 15, 30, 1000, and 3000 ns are analyzed to provide clarity on this point. PMID: 25734227
Deletion of exons 4 to 10 (NEMODelta4-10) accounts for about 80% of cases (familial and sporadic) of Incontinentia pigmenti. PMID: 26564087
results further reveal that cFLIPLrequires the linear ubiquitin chain assembly complex and the kinase TAK1 for activation of the IKK kinase PMID: 26865630
USP18 negatively regulates NF-kappaB signaling by targeting TAK1 and NEMO for deubiquitination through distinct mechanisms. PMID: 26240016
A missense mutation in IKBKG causes a Nager syndrome or an atypical incontinentia pigmenti phenotype. IKBKG mutations are typically associated with preterm male death, but this variant is associated with survival for 8-15 days. PMID: 25441681
Recruitment of A20 to the C-terminal domain of NEMO represents a novel mechanism limiting NF-kappaB activation by NEMO, and its absence results in autoinflammatory disease. PMID: 26802121
Authors show that Rab11-GMPPNP-FIP3-Rabin8 is more stable than Rab11-GMPPNP-Rabin8, owing to direct interaction between Rabin8 and FIP3 within the dual effector-bound complex. PMID: 26258637
Somatic mosaicism of a novel IKBKG nonsense mutation in a male patient with incontinentia pigmenti. PMID: 25944529
COMMD7's binding to NEMO does not interfere with the binding to the IKKs, and that the disruption of the IKK complex through the use of the NBP competitor impairs the termination of NF-kappaB activity PMID: 26060140
findings suggest that rare, functional variants in MYD88, IRAK4 or IKBKG do not significantly contribute to IPD susceptibility in adults at the population level PMID: 25886387
the incontinentia pigmenti patients presented a common IKBKG exon 4-10 deletion PMID: 24073555
A novel mutation, designated c.916G>A (p.D306N) is described. NEMO expression was unaffected, but ubiquitylation was decreased causing ectodermal dysplasia, immunodeficiency, incontinentia pigmenti, and immune thrombocytopenic purpura. PMID: 26117626
IKKgamma is a parallel coiled-coil whose response to binding of vFLIP or IKKbeta is localized twisting. PMID: 25979343
IPO3 binds NEMO, promotes its nuclear import, and is critical for DNA damage-dependent NF-kappaB activation. PMID: 26060253
unanchored polyubiquitin has a role in regulation by inducing NEMO conformational change by an allosteric mechanism PMID: 25866210
The stability of the NEMO coiled coil is maintained by strong interhelix interactions in the region centered on residue 54. PMID: 25400026
Mass spectrometric analysis demonstrated that WA covalently modifies NEMO on a cysteine residue within the C-terminal zinc finger (ZF) domain. Point mutations to the ZF can reverse the WA-induced Lys-48-polyubiquitin binding phenotype PMID: 25296760
NEMO patients without ectodermal dysplasia and anhidrosis have more robust immunologic responses. PMID: 24682681
the rescuing of the binding affinity implies that a preordered IKK-binding region of NEMO is compatible with IKK binding, and the conformational heterogeneity observed in NEMO(44-111) may be an artifact of the truncation. PMID: 25286246
IKBKG gene mutation was discovered as a cause for incontinentia pigmenti. (Meta-analysis) PMID: 23802866
We report the results of genomic analysis for a girl with incontinentia pigmenti, but without NEMO mutation PMID: 24487970
Data suggest the potential of targeting of Nemo-Like Kinase (NLK) to treat a range of tumourigenic conditions characterised by PTEN deficiency. PMID: 23144700
21 new point mutations have been reported, which further extend the spectrum of pathologic variants in Incontinentia pigmenti patients: premature stop codon, frameshift mutation, or a partial loss of NEMO/IKKgamma activity (splicing and missense). PMID: 24339369
p62 interacts with NEMO, the regulatory subunit of the complex responsible for activation of NF-kappaB transcription factor. PMID: 24270048
NEMO is essential for Kaposi's sarcoma-associated herpesvirus-encoded vFLIP K13-induced gene expression and protection against death receptor-induced cell death. PMID: 24672029
Identified is a post-translational modification of NEMO - phosphorylation of residue 387. Phosphorylation of serine 387 is not an absolute requirement for NF-kappaB signalling. PMID: 24012789
IKKgamma facilitates RhoA activation via a guanine nucletotide exchange factor, which in turn activates ROCK to phosphorylate IKKbeta, leading to NF-kappaB activation that induced the chemokine expression and cell migration upon TGF-beta1. PMID: 24240172
Data suggest that all seven cysteines (4 in zinc finger domain) of NEMO (NF-kappaB essential modulator protein) can be simultaneously mutated to alanine without affecting binding affinity of NEMO for I-kappa B kinase beta catalytic subunit. PMID: 24266532
USP10 inhibits genotoxic NF-kappaB activation by MCPIP1-facilitated deubiquitination of NEMO. PMID: 24270572
Merkel cell polyomavirus small T antigen targets the NEMO adaptor protein to disrupt inflammatory signaling. PMID: 24109239
NEMO ZF, like other NEMO related-ZFs, binds mono-Ub and di-Ub with distinct stoichiometries, indicating the presence of a new Ub site within the NEMO ZF. PMID: 24100029
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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)
Subcellular Location
Cytoplasm. Nucleus.
Tissue Specificity
Heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.