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https://www.readbyqxmd.com/read/29152796/the-genetic-basis-of-seborrheic-dermatitis-a-review
#1
REVIEW
Marko A Karakadze, Penelope A Hirt, Tongyu C Wikramanayake
Seborrheic Dermatitis (SD) is a common inflammatory skin disease that presents as itchy, flaking skin in the seborrheic areas. Various environmental and intrinsic factors have been identified as predisposing factors for SD, but its etiology remains poorly understood. Although it was recognized that genetic factors play a role in SD etiology, there have not been studies that systematically review the literature specifically for causal mutations or protein deficiencies in SD. In this review, we searched various databases for gene mutations and protein deficiencies that cause SD or SD-like phenotype in humans and experimental animals, and summarize 11 gene mutations or protein deficiencies that were described in the literature...
November 20, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29142404/chalepin-a-compound-from-ruta-angustifolia-l-pers-exhibits-cell-cycle-arrest-at-s-phase-suppresses-nuclear-factor-kappa-b-nf-%C3%AE%C2%BAb-pathway-signal-transducer-and-activation-of-transcription-3-stat3-phosphorylation-and-extrinsic-apoptotic-pathway-in-non-small-cell
#2
Jaime Stella Moses Richardson, Norhaniza Aminudin, Sri Nurestri Abd Malek
Background: Plants have been a major source of inspiration in developing novel drug compounds in the treatment of various diseases that afflict human beings worldwide. Ruta angustifolia L. Pers known locally as Garuda has been conventionally used for various medicinal purposes such as in the treatment of cancer. Objective: A dihydrofuranocoumarin named chalepin, which was isolated from the chloroform extract of the plant, was tested on its ability to inhibit molecular pathways of human lung carcinoma (A549) cells...
October 2017: Pharmacognosy Magazine
https://www.readbyqxmd.com/read/29142064/the-resurrection-of-the-piddosome-emerging-roles-in-the-dna-damage-response-and-centrosome-surveillance
#3
REVIEW
Valentina Sladky, Fabian Schuler, Luca L Fava, Andreas Villunger
The PIDDosome is often used as the alias for a multi-protein complex that includes the p53-induced death domain protein 1 (PIDD1), the bipartite linker protein CRADD (also known as RAIDD) and the pro-form of an endopeptidase belonging to the caspase family, i.e. caspase-2. Yet, PIDD1 variants can also interact with a number of other proteins that include RIPK1 (also known as RIP1) and IKBKG (also known as NEMO), PCNA and RFC5, as well as nucleolar components such as NPM1 or NCL. This promiscuity in protein binding is facilitated mainly by autoprocessing of the full-length protein into various fragments that contain different structural domains...
November 15, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/29128367/nemo-links-nuclear-factor-%C3%AE%C2%BAb-to-human-diseases
#4
REVIEW
Gunter Maubach, Michael Naumann
The nuclear factor (NF)-κB essential modulator (NEMO) is a key regulator in NF-κB-mediated signaling. By transmitting extracellular or intracellular signals, NEMO can control NF-κB-regulated genes. NEMO dysfunction is associated with inherited diseases such as incontinentia pigmenti (IP), ectodermal dysplasia, anhidrotic, with immunodeficiency (EDA-ID), and some cancers. We focus on molecular studies, human case reports, and mouse models emphasizing the significance of NEMO molecular interactions and modifications in health and diseases...
November 8, 2017: Trends in Molecular Medicine
https://www.readbyqxmd.com/read/29125880/mavs-activates-tbk1-and-ikk%C3%AE%C2%B5-through-trafs-in-nemo-dependent-and-independent-manner
#5
Run Fang, Qifei Jiang, Xiang Zhou, Chenguang Wang, Yukun Guan, Jianli Tao, Jianzhong Xi, Ji-Ming Feng, Zhengfan Jiang
Mitochondrial antiviral-signaling protein (MAVS) transmits signals from RIG-I-like receptors after RNA virus infections. However, the mechanism by which MAVS activates downstream components, such as TBK1 and IKKα/β, is unclear, although previous work suggests the involvement of NEMO or TBK1-binding proteins TANK, NAP1, and SINTBAD. Here, we report that MAVS-mediated innate immune activation is dependent on TRAFs, partially on NEMO, but not on TBK1-binding proteins. MAVS recruited TBK1/IKKε by TRAFs that were pre-associated with TBK1/IKKε via direct interaction between the coiled-coil domain of TRAFs and the SDD domain of TBK1/IKKε...
November 10, 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/29122559/constant-hepatic-atp-concentrations-during-prolonged-fasting-and-absence-of-effects-of-cerbomed-nemos-%C3%A2-on-parasympathetic-tone-and-hepatic-energy-metabolism
#6
Sofiya Gancheva, Alessandra Bierwagen, Daniel F Markgraf, Gidon J Bönhof, Kevin G Murphy, Erifili Hatziagelaki, Jesper Lundbom, Dan Ziegler, Michael Roden
OBJECTIVE: Brain insulin-induced improvement in glucose homeostasis has been proposed to be mediated by the parasympathetic nervous system. Non-invasive transcutaneous auricular vagus nerve stimulation (taVNS) activating afferent branches of the vagus nerve may prevent hyperglycemia in diabetes models. We examined the effects of 14-min taVNS vs sham stimulation by Cerbomed Nemos(®) on glucose metabolism, lipids, and hepatic energy homeostasis in fasted healthy humans (n = 10, age 51 ± 6 yrs, BMI 25...
October 25, 2017: Molecular Metabolism
https://www.readbyqxmd.com/read/29111346/evidence-for-m1-linked-polyubiquitin-mediated-conformational-change-in-nemo
#7
Arthur V Hauenstein, Guozhou Xu, Venkataraman Kabaleeswaran, Hao Wu
The NF-κB essential modulator (NEMO) is the scaffolding subunit of the inhibitor of κB kinase (IKK) holocomplex and is required for the activation of the catalytic IKK subunits, IKKα and IKKβ, during the canonical inflammatory response. Although structures of shorter constructs of NEMO have been solved, efforts to elucidate the full-length structure of NEMO have proved difficult due to its apparent high conformational plasticity. To better characterize the gross dimensions of full-length NEMO, we employed in-line size exclusion chromatography-small-angle X-ray scattering...
October 27, 2017: Journal of Molecular Biology
https://www.readbyqxmd.com/read/29069608/uev1a-ubc13-catalyzes-k63-linked-ubiquitination-of-rhbdf2-to-promote-tace-maturation
#8
Yiran Zhang, Yadan Li, Xiaoran Yang, Juanjuan Wang, Ruifeng Wang, Xianghao Qian, Weiwei Zhang, Wei Xiao
The TNFα-induced NF-κB signaling pathway plays critical roles in multiple biological processes. Extensive studies have explored the mechanisms regulating this signaling cascade, and identified an E2 complex, Uev1A-Ubc13, that mediates K63-linked poly-Ub chain formation and thus recruits NEMO to activate the signaling transduction. In this study, we demonstrate that the Uev1A-Ubc13 complex simultaneously serves as a repressor of the NF-κB pathway. It was found that cells overexpressing UEV1A silence the signal cascade earlier than control cells...
October 22, 2017: Cellular Signalling
https://www.readbyqxmd.com/read/29065166/genetic-diversity-and-connectivity-in-the-east-african-giant-mud-crab-scylla-serrata-implications-for-fisheries-management
#9
Cyrus Rumisha, Filip Huyghe, Diary Rapanoel, Nemo Mascaux, Marc Kochzius
The giant mud crab Scylla serrata provides an important source of income and food to coastal communities in East Africa. However, increasing demand and exploitation due to the growing coastal population, export trade, and tourism industry are threatening the sustainability of the wild stock of this species. Because effective management requires a clear understanding of the connectivity among populations, this study was conducted to assess the genetic diversity and connectivity in the East African mangrove crab S...
2017: PloS One
https://www.readbyqxmd.com/read/29037783/suppression-of-nemo-like-kinase-by-mir-71-in-echinococcus-multilocularis
#10
Xiaola Guo, Xueyong Zhang, Jing Yang, Xiaoliang Jin, Juntao Ding, Haitao Xiang, Mazhar Ayaz, Xuenong Luo, Yadong Zheng
Echinococcus multilocularis metacestodes are a causative pathogen for alveolar echinococcosis in human beings, and have been found to express miRNAs including emu-miR-71. miR-71 is evolutionarily conserved and highly expressed across platyhelminths, but little is known about its role. Here it was shown that emu-miR-71 was differentially expressed in protoscoleces and was unlikely to be expressed in neoblasts. The results of the luciferase assay indicated that emu-miR-71 was able to bind in vitro to the 3'-UTR of emu-nlk, encoding a key regulator of cell division, causing significant downregulation of luciferase activity (p < 0...
October 13, 2017: Experimental Parasitology
https://www.readbyqxmd.com/read/28993958/functional-evaluation-of-an-ikbkg-variant-suspected-to-cause-immunodeficiency-without-ectodermal-dysplasia
#11
Glynis Frans, Jutte van der Werff Ten Bosch, Leen Moens, Rik Gijsbers, Majid Changi-Ashtiani, Hassan Rokni-Zadeh, Mohammad Shahrooei, Greet Wuyts, Isabelle Meyts, Xavier Bossuyt
Hypomorphic IKBKG mutations in males are typically associated with anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID). Some mutations cause immunodeficiency without EDA (NEMO-ID). The immunological profile associated with these NEMO-ID variants is not fully documented. We present a 2-year-old patient with suspected immunodeficiency in which a hemizygous p.Glu57Lys IKBKG variant was identified. At the age of 1 year, he had an episode of otitis media that evolved into a bilateral mastoiditis (Pseudomonas spp)...
November 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28976986/investigating-the-impact-of-media-on-demand-for-wildlife-a-case-study-of-harry-potter-and-the-uk-trade-in-owls
#12
Diane A Megias, Sean C Anderson, Robert J Smith, Diogo Veríssimo
In recent decades, a substantial number of popular press articles have described an increase in demand for certain species in the pet trade due to films such as "Finding Nemo", "Ninja turtles", and "Harry Potter". Nevertheless, such assertions are largely supported only by anecdotal evidence. Given the role of the wildlife trade in the spread of pathogens and zoonosis, the introduction of invasive species, the overexploitation of biodiversity, and the neglect of animal welfare, it is crucial to understand what factors drive demand for a species...
2017: PloS One
https://www.readbyqxmd.com/read/28974699/numbl-interacts-with-tak1-traf6-and-nemo-to-negatively-regulate-nf-%C3%AE%C2%BAb-signaling-during-osteoclastogenesis
#13
Gaurav Swarnkar, Tim Hung-Po Chen, Manoj Arra, Amjad M Nasir, Gabriel Mbalaviele, Yousef Abu-Amer
NF-κB signaling is essential for osteoclast differentiation and skeletal homeostasis. We have reported recently that NUMB-like (NUMBL) protein modulates osteoclastogenesis by down regulating NF-κB activation. Herein, we decipher the mechanism underlying this phenomenon. We found that whereas NUMBL mRNA expression decreases upon stimulation of wild type (WT) bone marrow macrophages (BMMs) with RANKL, TAK1 deficiency in these cells leads to increased NUMBL and decreased TRAF6 and NEMO expression. These changes were restored upon WT-TAK1 expression, but not with catalytically inactive TAK1-K63W, suggesting that TAK1 enzymatic activity is required for these events...
October 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28951128/nemo-peptide-inhibits-the-growth-of-pancreatic-ductal-adenocarcinoma-by-blocking-nf-%C3%AE%C2%BAb-activation
#14
Zhuonan Zhuang, Hao Li, Harold Lee, Mitzi Aguilar, Takashi Gocho, Huaiqiang Ju, Tomonori Iida, Jianhua Ling, Jie Fu, Min Wu, Yichen Sun, Yu Lu, Paul J Chiao
NF-κB essential modulator (NEMO) binds and regulates IκB kinase (IKK) and is required for NF-κB activation. The NEMO-binding domain peptide (NBDP) of IKK was found to inhibit NF-κB activation and promote apoptosis in cancer cells. Studies have shown that constitutive NF-κB activation, one of the signature molecular alterations in pancreatic ductal adenocarcinoma (PDAC), is a potential therapeutic target. However, preclinical and therapeutic evidence that supports direct targeting of IKK activation in therapy is lacking...
September 23, 2017: Cancer Letters
https://www.readbyqxmd.com/read/28939760/nemo-ikk%C3%AE-are-essential-for-irf3-and-nf-%C3%AE%C2%BAb-activation-in-the-cgas-sting-pathway
#15
Run Fang, Chenguang Wang, Qifei Jiang, Mengze Lv, Pengfei Gao, Xiaoyu Yu, Ping Mu, Rui Zhang, Sheng Bi, Ji-Ming Feng, Zhengfan Jiang
Cytosolic dsDNA activates the cyclic GMP-AMP synthase (cGAS)-stimulator of IFN genes (STING) pathway to produce cytokines, including type I IFNs. The roles of many critical proteins, including NEMO, IKKβ, and TBK1, in this pathway are unclear because of the lack of an appropriate system to study. In this article, we report that lower FBS concentrations in culture medium conferred high sensitivities to dsDNA in otherwise unresponsive cells, whereas higher FBS levels abrogated this sensitivity. Based on this finding, we demonstrated genetically that NEMO was critically involved in the cGAS-STING pathway...
November 1, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28935643/transplant-for-nemo-this-and-much-much-more
#16
Dennis D Hickstein, Luigi Notarangelo
No abstract text is available yet for this article.
September 21, 2017: Blood
https://www.readbyqxmd.com/read/28932485/incontinentia-pigmenti-in-a-child-with-suspected-retinoblastoma
#17
Stephanie J Weiss, Archana Srinivasan, Michael A Klufas, Carol L Shields
BACKGROUND: Incontinentia pigmenti is a rare X-linked dominant syndrome caused by mutation in the NEMO/IKKgamma gene, and characterized by a spectrum of cutaneous, ocular, neurologic and dental abnormalities. In the eye, findings include retinal vascular non-perfusion, occasionally with traction retinal detachment, retinal fibrosis, and retinal pigment epithelium defects. These findings can resemble retinoblastoma, especially when vitreoretinal fibrosis produces leukocoria. CASE REPORT: A 2-month-old girl born full-term presented with leukocoria, suspicious for retinoblastoma...
2017: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/28931678/ikk%C3%AE-mimetic-peptides-block-the-resistance-to-apoptosis-associated-with-kaposi-s-sarcoma-associated-herpesvirus-infection
#18
Louise C Briggs, A W Edith Chan, Christopher A Davis, Nicholas Whitelock, Hajira A Hotiana, Mehdi Baratchian, Claire Bagnéris, David L Selwood, Mary K Collins, Tracey E Barrett
Primary effusion lymphoma (PEL) is a lymphogenic disorder associated with Kaposi's sarcoma-associated herpesvirus (KSHV) infection. Key to the survival and proliferation of PEL is the canonical NF-κB pathway, which becomes constitutively activated following overexpression of the viral oncoprotein KSHV vFLIP (ks-vFLIP). This arises from its capacity to form a complex with the modulatory subunit of the IκB kinase (IKK) kinase, IKKγ (or NEMO), resulting in the overproduction of proteins that promote cellular survival and prevent apoptosis, both of which are important drivers of tumorigenesis...
December 1, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28919996/mek-inhibition-and-immune-responses-in-advanced-melanoma
#19
REVIEW
Reinhard Dummer, Egle Ramelyte, Sabrina Schindler, Olaf Thürigen, Mitchell P Levesque, Peter Koelblinger
phase II and III clinical trials demonstrated modest anti- tumor activity of Binimetinib (MEK162) - a potent allosteric inhibitor of MEK1 and MEK2- in patients with advanced NRAS mutant melanoma. The analysis of the NEMO study in NRAS mutated melanoma, has shown that pre-treatment with immunotherapy improved the outcome of binimetinib therapy. We discuss this finding in the context of in vitro and in vivo effects of MEK inhibition on immuno-critical pathways and interactions.
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28917830/al-awadi-raas-rothschild-syndrome-with-dental-anomalies-and-a-novel-wnt7a-mutation
#20
Piranit Nik Kantaputra, Seema Kapoor, Prashant Verma, Massupa Kaewgahya, Katsushige Kawasaki, Atsushi Ohazama, James R Ketudat Cairns
Al-Awadi-Raas-Rothschild syndrome (AARRS; OMIM 276820) is a very rare autosomal recessive limb malformation syndrome caused by WNT7A mutations. AARRS is characterized by various degrees of limb aplasia and hypoplasia. Normal intelligence and malformations of urogenital system are frequent findings. Complete loss of WNT7A function has been shown to cause AARRS, however, its partial loss leads to the milder malformation, Fuhrmann syndrome. An Indian boy affected with AARRS is reported. A novel homozygous base substitution mutation c...
September 14, 2017: European Journal of Medical Genetics
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