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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
NEMO Prevents Steatohepatitis and Hepatocellular Carcinoma by Inhibiting RIPK1 Kinase Activity-Mediated Hepatocyte Apoptosis.
Pasparakis et al., Köln, Germany. In Cancer Cell, 09 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.
Clinical study of 20 patients with incontinentia pigmenti.
Kiszewski et al., Porto Alegre, Brazil. In Int J Dermatol, 06 Dec 2015
BACKGROUND: Incontinentia pigmenti (IP) is a rare genodermatosis with early prenatal lethality in affected males.
Molecular Pathways: Fatty Acid Synthase.
Infante et al., United States. In Clin Cancer Res, 30 Nov 2015
A new generation of molecules including GSK 2194069, JNJ-54302833, IPI-9119, and TVB-2640 are in development, but only TVB-2640 has moved into the clinic.
NF-κB contributes to MMP1 expression in breast cancer spheroids causing paracrine PAR1 activation and disintegrations in the lymph endothelial barrier in vitro.
Krupitza et al., Vienna, Austria. In Oncotarget, 15 Nov 2015
UNASSIGNED: RELA, RELB, CREL, NFKB1 and NFKB2, and the upstream regulators NEMO and NIK were knocked-down in lymph endothelial cells (LECs) and in MDA-MB231 breast cancer spheroids to study the contribution of NF-κB in vascular barrier breaching.
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, 12 Nov 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.
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, 10 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).
Cloning and functional characterization of an isopentenyl diphosphate isomerase gene from Tripterygium wilfordii.
Huang et al., Beijing, China. In Biotechnol Appl Biochem, Sep 2015
In the present study, a full-length cDNA encoding IPI (designate as TwIPI, GenBank accession no.KT279355) was cloned from a suspension of cultured cells from T. wilfordii.
Treatment approach to newly diagnosed diffuse large B-cell lymphoma.
Salles et al., Lyon, France. In Semin Hematol, Apr 2015
High prognostic heterogeneity is observed among patients with DLBCL with long-term survival ranging from 30% to more than 90% according to clinic-biological prognostics such as the International Prognostic Index (IPI) and its subsequent revised variants.
Molecular pathways: novel approaches for improved therapeutic targeting of Hedgehog signaling in cancer stem cells.
Fields et al., Jacksonville, United States. In Clin Cancer Res, Mar 2015
However, to date, Hh inhibitors, specifically those targeting Smoothened [such as vismodegib, BMS-833923, saridegib (IPI-926), sonidegib/erismodegib (LDE225), PF-04449913, LY2940680, LEQ 506, and TAK-441], have demonstrated good efficacy as monotherapy in patients with basal cell carcinoma and medulloblastoma, but have shown limited activity in other tumor types.
Simultaneous Overexpression of Functional Human HO-1, E5NT and ENTPD1 Protects Murine Fibroblasts against TNF-α-Induced Injury In Vitro.
Giovannoni et al., Monza, Italy. In Plos One, Dec 2014
Moreover, a gene expression analyses demonstrated that the expression of the three genes has a role in modulating key regulators of TNF-α signalling pathway, namely Nemo and Tnfaip3, that promoted pro-survival phenotype in TNF-α injured cells.
VEGF overexpression is a valuable prognostic factor for non-Hodgkin's lymphoma evidence from a systemic meta-analysis.
Chai et al., Hangzhou, China. In Dis Markers, Dec 2014
Additionally, VEGF overexpression did not correlate with performance status, LDH level, IPI score, tumor staging, B symptoms, or NHL relapse.
Management of diffuse large B-cell lymphoma (DLBCL).
Pfreundschuh et al., Homburg, Germany. In Cancer Treat Res, Dec 2014
Treatment is usually tailored according to the individual risk profile of a DLBCL patient according to the International Prognostic Index (IPI).
The paracaspase MALT1 cleaves HOIL1 reducing linear ubiquitination by LUBAC to dampen lymphocyte NF-κB signalling.
Overall et al., Vancouver, Canada. In Nat Commun, Dec 2014
Late in the NF-κB activation cycle HOIL1 cleavage transiently reduces linear ubiquitination, including of NEMO and RIP1, dampening NF-κB activation and preventing reactivation.
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, Jul 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.
Histone methyltransferase Ash1l suppresses interleukin-6 production and inflammatory autoimmune diseases by inducing the ubiquitin-editing enzyme A20.
Cao et al., Hangzhou, China. In Immunity, 2013
Ash1l suppressed NF-κB, mitogen-activated protein kinase (MAPK) pathways, and subsequent IL-6 production via facilitating A20-mediated NF-κB signal modulator NEMO and transducer TRAF6 deubiquitination.
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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