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Inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma

Incontinentia Pigmenti, NEMO, IPI, IKKgamma, IP2
This gene encodes the regulatory subunit of the inhibitor of kappaB kinase (IKK) complex, which activates NF-kappaB resulting in activation of genes involved in inflammation, immunity, cell survival, and other pathways. Mutations in this gene result in incontinentia pigmenti, hypohidrotic ectodermal dysplasia, and several other types of immunodeficiencies. Multiple transcript variants encoding different isoforms have been found for this gene. A pseudogene highly similar to this locus is located in an adjacent region of the X chromosome. [provided by RefSeq, Aug 2011] (from NCBI)
Top mentioned proteins: CAN, NF-kappaB, HAD, Ubiquitin, V1a
Papers using Incontinentia Pigmenti antibodies
Bioinformatics enrichment tools: paths toward the comprehensive functional analysis of large gene lists
Martelli Fabio, In PLoS ONE, 2008
... The Refseq ids were converted to IPI ids using an online server ...
Control of canonical NF-κB activation through the NIK-IKK complex pathway
May Michael J. et al., In Oncogene, 2007
... Anti-RET, IKKα, p50, NIK, TRAF3, TRAF2, RelB, and NEMO were from Santa Cruz Biotechnology (Santa Cruz, CA) ...
Papers on Incontinentia Pigmenti
NF-kB Essential MOdulator (NEMO) Is Critical for Thyroid Function.
Stilo et al., Italy. In J Biol Chem, Feb 2016
UNASSIGNED: The I-kB kinase (IKK) subunit NEMO/IKKg; (NEMO) is an adapter molecule that is critical for canonical activation of NF-kB, a pleiotropic transcription factor controlling immunity, differentiation, cell growth, tumorigenesis and apoptosis.
Non-canonical pathway network modelling and ubiquitination site prediction through homology modelling of NF-κB.
Febin Prabhu Dass et al., Vellore, India. In Gene, Feb 2016
Both the pathways involve ubiquitination of its repressors, that is to say ubiquitination of I-κB by NEMO/IKK-α/IKK-β complex in case of NF-κB1 and that of p100 by IKK-α homodimer in case of NF-κB2.
Novel Non-Peptide Small Molecules Preventing IKKβ/NEMO Association Inhibit NF-κB Activation in LPS-Stimulated J774 Macrophages.
Lavecchia et al., Napoli, Italy. In Biochem Pharmacol, Feb 2016
Association of the IKK complex relies on critical interactions between the C-terminus NBD (NEMO binding domain) of the catalytic subunits IKKα and IKKβ, and the regulatory subunit NEMO (NF-κB Essential Modulator).
Loss-of-function mutations in TNFAIP3 leading to A20 haploinsufficiency cause an early-onset autoinflammatory disease.
Aksentijevich et al., Bethesda, United States. In Nat Genet, Jan 2016
In cells expressing mutant A20 protein, there is defective removal of Lys63-linked ubiquitin from TRAF6, NEMO and RIP1 after stimulation with tumor necrosis factor (TNF).
Expression and significance of Ku80 and PDGFR-α in nasal NK/T-cell lymphoma.
Zhong et al., Davao, Philippines. In Pathol Res Pract, Jan 2016
Ku80 and PDGFR-α expressions were not correlated with patient's gender, age, B symptoms, LDH, Ann Arbor stage, IPI score and treatment (P>0.05).
Effectiveness of Corticosteroid Therapy for Acute Neurological Symptoms in Incontinentia Pigmenti.
Itani et al., Yokohama, Japan. In Pediatr Neurol, Jan 2016
BACKGROUND: Incontinentia pigmenti is a rare neurocutaneous disorder that may present neurological symptoms, in addition to characteristic skin rashes.
Molecular Pathways: Fatty Acid Synthase.
Infante et al., Nashville, United States. In Clin Cancer Res, Jan 2016
A new generation of molecules, including GSK2194069, JNJ-54302833, IPI-9119, and TVB-2640, are in development, but only TVB-2640 has moved into the clinic.
The emerging role of Nemo-like kinase (NLK) in the regulation of cancers.
Li et al., Hefei, China. In Tumour Biol, Dec 2015
Nemo-like kinase (NLK) is an evolutionarily conserved mitogen-activated protein (MAP) kinase-related kinase that is highly expressed in neural tissues and minimally detected in others.
Mosaic Neurocutaneous Disorders and Their Causes.
Praticò et al., Catania, Italy. In Semin Pediatr Neurol, Dec 2015
In this article we will focus on the well known, and less defined mosaic neurocutaneous phenotypes and their related molecular/genetic bases, including the mosaic neurofibromatoses and their related forms (ie, spinal neurofibromatosis and schwannomatosis); Legius syndrome; segmental arrangements in tuberous sclerosis; Sturge-Weber and Klippel-Trenaunay syndromes; microcephaly/megalencephaly-capillary malformation; blue rubber bleb nevus syndrome; Wyburn-Mason syndrome; mixed vascular nevus syndrome; PHACE syndrome; Incontinentia pigmenti; pigmentary mosaicism of the Ito type; neurocutaneous melanosis; cutis tricolor; speckled lentiginous syndrome; epidermal nevus syndromes; Becker's nevus syndrome; phacomatosis pigmentovascularis and pigmentokeratotica; Proteus syndrome; and encephalocraniocutaneous lipomatosis.
Genome-Wide Association Study of Event-Free Survival in Diffuse Large B-Cell Lymphoma Treated With Immunochemotherapy.
Cerhan et al., Bordeaux, France. In J Clin Oncol, Dec 2015
In exploratory analyses, a two-single nucleotide polymorphism risk score was highly predictive of EFS (P = 1.78 × 10(-12)) and was independent of treatment, IPI, and cell-of-origin classification.
NEMO Prevents Steatohepatitis and Hepatocellular Carcinoma by Inhibiting RIPK1 Kinase Activity-Mediated Hepatocyte Apoptosis.
Pasparakis et al., Köln, Germany. In Cancer Cell, Dec 2015
IκB kinase/necrosis factor κB (IKK/NF-κB) signaling exhibits important yet opposing functions in hepatocarcinogenesis. Mice lacking NEMO in liver parenchymal cells (LPC) spontaneously develop steatohepatitis and hepatocellular carcinoma (HCC) suggesting that NF-κB prevents liver disease and cancer.
Novel Therapies for Chronic Lymphocytic Leukemia: A Canadian Perspective.
Sehn et al., Calgary, Canada. In Clin Lymphoma Myeloma Leuk, Nov 2015
Two groups of novel therapeutic agents-anti-CD20 monoclonal antibodies and small molecule inhibitors-are attempting to address these issues, and 5 of these agents have progressed to phase 3 trials: obinutuzumab, idelalisib, ibrutinib, venetoclax (ABT-199), and duvelisib (IPI-145).
Prognostic Significance of Diffuse Large B-Cell Lymphoma Cell of Origin Determined by Digital Gene Expression in Formalin-Fixed Paraffin-Embedded Tissue Biopsies.
Gascoyne et al., Vancouver, Canada. In J Clin Oncol, Oct 2015
PURPOSE: To evaluate the prognostic impact of cell-of-origin (COO) subgroups, assigned using the recently described gene expression-based Lymph2Cx assay in comparison with International Prognostic Index (IPI) score and MYC/BCL2 coexpression status (dual expressers).
Incontinentia pigmenti (Bloch-Sulzberger syndrome).
Narayanan et al., New Orleans, United States. In Handb Clin Neurol, 2014
IP is caused by mutation of the IKBKG/NEMO gene on Xq28.
Rhbdd3 controls autoimmunity by suppressing the production of IL-6 by dendritic cells via K27-linked ubiquitination of the regulator NEMO.
Cao et al., Shanghai, China. In Nat Immunol, 2014
Rhbdd3-deficient mice spontaneously developed autoimmune diseases characterized by an increased abundance of the TH17 subset of helper T cells and decreased number of regulatory T cells due to the increase in IL-6 from DCs. Rhbdd3 directly bound to Lys27 (K27)-linked polyubiquitin chains on Lys302 of the modulator NEMO (IKKγ) via the ubiquitin-binding-association (UBA) domain in endosomes.
NEMO ensures signaling specificity of the pleiotropic IKKβ by directing its kinase activity toward IκBα.
Hoffmann et al., San Diego, United States. In Mol Cell, 2012
Data show that NEMO forms a complex with IKKbeta and IkappaBalpha.
Analysis of nuclear factor-κB (NF-κB) essential modulator (NEMO) binding to linear and lysine-linked ubiquitin chains and its role in the activation of NF-κB.
Dikic et al., Frankfurt am Main, Germany. In J Biol Chem, 2012
Data indicate that modification of NEMO with linear di-ubiquitin is sufficient for full NF-kappaB activation.
NEMO expression in human hepatocellular carcinoma and its association with clinical outcome.
Pichler et al., Graz, Austria. In Hum Pathol, 2012
Although low NEMO expression is correlated with a poor 5-year overall survival in patients with hepatocellular carcinoma, NEMO cannot be regarded as an independent prognostic marker for predicting the clinical outcome of patients suffering from HCC.
Frequent somatic mosaicism of NEMO in T cells of patients with X-linked anhidrotic ectodermal dysplasia with immunodeficiency.
Heike et al., Kyoto, Japan. In Blood, 2012
These data indicate that the frequency of somatic mosaicism of NEMO is high in X-linked anhidrotic ectodermal dysplasia with immunodeficiency patients.
Viral mediated redirection of NEMO/IKKγ to autophagosomes curtails the inflammatory cascade.
Brune et al., Hamburg, Germany. In Plos Pathog, 2012
Murine cytomegalovirus inhibits the inflammatory cascade by blocking Toll-like receptor and IL-1 receptor-dependent NF-kappaB activation through an interaction of the viral M45 protein with the NF-kappaB essential modulator.
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