Anti-IKBKG Antibody (R4A25)

Catalog No.

RHN62003

Species reactivity

Human

Host species

Mouse

Isotype

IgG2a

Clonality

Monoclonal

Tested applications

IHC: 1:100-1:200, IP: 1:100-1:200, WB: 1:500-1:1000

Target

Inhibitor of nuclear factor kappa-B kinase subunit gamma, NEMO, IKKAP1, IKK-gamma, IkB kinase-associated protein 1, IkB kinase subunit gamma, FIP-3, NF-kappa-B essential modifier, NF-kappa-B essential modulator, IKBKG, I-kappa-B kinase subunit gamma, FIP3, IKKG

Concentration

1 mg/ml

Endotoxin level

Please contact with the lab for this information.

Purity

>95% as determined by SDS-PAGE.

Purification

Protein A/G purified from cell culture supernatant.

Accession

Q9Y6K9

Applications

IHC, IP, WB

Form

Liquid

Storage buffer

0.01M PBS, pH 7.4, 0.05% Sodium Azide.

Stability and Storage

Use a manual defrost freezer and avoid repeated freeze-thaw cycles. Store at 4°C short term (1-2 weeks). Store at -20°C 12 months. Store at -80°C long term.

Clone ID

R4A25

Identification of soluble biomarkers that associate with distinct manifestations of long COVID., PMID:40307449

NF-ΚB Activation as a Key Driver in Chronic Lymphocytic Leukemia Evolution to Richter's Syndrome: Unraveling the Influence of Immune Microenvironment Dynamics., PMID:39596634

Finding NEMO in the thymus., PMID:39432904

Incontinentia pigmenti underlies thymic dysplasia, autoantibodies to type I IFNs, and viral diseases., PMID:39352576

Role of Skewed X-Chromosome Inactivation in Common Variable Immunodeficiency., PMID:38265673

Genome-wide association studies for immunoglobulin concentrations in colostrum and serum in Chinese Holstein., PMID:35012443

NeMO mutations: a rare cause of monogenic Behçet-like disease., PMID:32954429

Improving the diagnostic efficiency of primary immunodeficiencies with targeted next-generation sequencing., PMID:32531373

Successful Allogeneic Stem Cell Transplantation in Nuclear Factor-Kappa B Essential Modulator Deficiency Syndrome After Treosulfan-Based Conditioning: A Case Report., PMID:32035679

T Cell Impairment Is Predictive for a Severe Clinical Course in NEMO Deficiency., PMID:31965418

Infliximab therapy for inflammatory colitis in an infant with NEMO deficiency., PMID:31713830

Viral antigens elicit augmented immune responses in primary Sjögren's syndrome., PMID:31665501

Placental Expression of NEMO Protein in Normal Pregnancy and Preeclampsia., PMID:30723530

Cell-Permeable Bicyclic Peptidyl Inhibitors against NEMO-IκB Kinase Interaction Directly from a Combinatorial Library., PMID:30176143

Intravitreal injection of ranibizumab in severe retinopathy of incontinentia pigmenti., PMID:29630932

Functional Evaluation of an IKBKG Variant Suspected to Cause Immunodeficiency Without Ectodermal Dysplasia., PMID:28993958

The paracaspase MALT1 cleaves HOIL1 reducing linear ubiquitination by LUBAC to dampen lymphocyte NF-κB signalling., PMID:26525107

Immunodeficiency among children with recurrent invasive pneumococcal disease., PMID:25831419

Immunoblot analysis of linear polyubiquitination of NEMO., PMID:25736756

BCL10 regulates RNF8/RNF168-mediated ubiquitination in the DNA damage response., PMID:24732096

Diagnosis and treatment in anhidrotic ectodermal dysplasia with immunodeficiency., PMID:22635013

Successful treatment with infliximab for inflammatory colitis in a patient with X-linked anhidrotic ectodermal dysplasia with immunodeficiency., PMID:21993693

Disseminated BCG infection mimicking metastatic nasopharyngeal carcinoma in an immunodeficient child with a novel hypomorphic NEMO mutation., PMID:21755389

Congenital alterations of NEMO glutamic acid 223 result in hypohidrotic ectodermal dysplasia and immunodeficiency with normal serum IgG levels., PMID:21704885

Tax ubiquitylation and SUMOylation control the dynamic shuttling of Tax and NEMO between Ubc9 nuclear bodies and the centrosome., PMID:20959607

A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency., PMID:20652909

A novel missense mutation in the nuclear factor-κB essential modulator (NEMO) gene resulting in impaired activation of the NF-κB pathway and a unique clinical phenotype presenting as MRSA subdural empyema., PMID:20652730

Allogeneic hematopoietic stem cell transplantation for X-linked ectodermal dysplasia and immunodeficiency: case report and review of outcomes., PMID:19225723

ATM mediates constitutive NF-kappaB activation in high-risk myelodysplastic syndrome and acute myeloid leukemia., PMID:19079347

IKBKG (nuclear factor-kappa B essential modulator) mutation can be associated with opportunistic infection without impairing Toll-like receptor function., PMID:18179816

Induction of human monocyte interleukin (IL)-8 by fibrinogen through the toll-like receptor pathway., PMID:17624583

IRAK4 and NEMO mutations in otherwise healthy children with recurrent invasive pneumococcal disease., PMID:16950813

Transient hemophagocytosis with deficient cellular cytotoxicity, monoclonal immunoglobulin M gammopathy, increased T-cell numbers, and hypomorphic NEMO mutation., PMID:16636116

Novel splicing mutation in the NEMO (IKK-gamma) gene with severe immunodeficiency and heterogeneity of X-chromosome inactivation., PMID:16333836

Specific NEMO mutations impair CD40-mediated c-Rel activation and B cell terminal differentiation., PMID:15578091

Nuclear factor kappaB essential modulator-deficient child with immunodeficiency yet without anhidrotic ectodermal dysplasia., PMID:15577852

Document Download

Anti-IKBKG Antibody (R4A25).pdf