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https://www.readbyqxmd.com/read/28628231/gene-therapy-decreases-seizures-in-a-model-of-incontinentia-pigmenti
#1
Godwin K Dogbevia, Kathrin Töllner, Jakob Körbelin, Sonja Bröer, Dirk A Ridder, Hanna Grasshoff, Claudia Brandt, Jan Wenzel, Beate K Straub, Martin Trepel, Wolfgang Löscher, Markus Schwaninger
OBJECTIVE: Incontinentia pigmenti (IP) is a genetic disease leading to severe neurological symptoms, such as epileptic seizures, but no specific treatment is available. IP is caused by pathogenic variants that inactivate the Nemo gene. Replacing Nemo through gene therapy might provide therapeutic benefits. METHODS: In a mouse model of IP we administered a single intravenous dose of the AAV vector AAV-BR1-CAG-NEMO delivering the Nemo gene to the brain endothelium...
June 19, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28626608/c-kit-modifies-the-inflammatory-status-of-smooth-muscle-cells
#2
Lei Song, Laisel Martinez, Zachary M Zigmond, Diana R Hernandez, Roberta M Lassance-Soares, Guillermo Selman, Roberto I Vazquez-Padron
BACKGROUND: c-Kit is a receptor tyrosine kinase present in multiple cell types, including vascular smooth muscle cells (SMC). However, little is known about how c-Kit influences SMC biology and vascular pathogenesis. METHODS: High-throughput microarray assays and in silico pathway analysis were used to identify differentially expressed genes between primary c-Kit deficient (Kit(W/W-v)) and control (Kit(+/+)) SMC. Quantitative real-time RT-PCR and functional assays further confirmed the differences in gene expression and pro-inflammatory pathway regulation between both SMC populations...
2017: PeerJ
https://www.readbyqxmd.com/read/28623244/it-s-a-cod-finding-nemo-impacted-fishbone-in-the-emergency-department
#3
Aileen McCabe, Andrew Patton, Nigel Salter
A 23-year-old woman presented to the emergency department (ED) with a sensation of a 'fish bone' stuck in her throat after eating cod. On physical examination, while she reported an uncomfortable sensation in her throat, no airway compromise was evident. Clinical examination, including ear, nose and throat (ENT) and oropharyngeal assessment, was unremarkable. A linear opacity consistent with a fishbone was visualised on a soft tissue lateral neck X-ray anterior to the vertebral body of C4-6. One attempt to visualise the fishbone on direct laryngoscopy failed in the ED...
June 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28610600/the-cumulative-number-of-micro-haemorrhages-and-micro-thromboses-in-nailfold-videocapillaroscopy-is-a-good-indicator-of-disease-activity-in-systemic-sclerosis-a-validation-study-of-the-nemo-score
#4
Romina Andracco, Rosaria Irace, Eleonora Zaccara, Serena Vettori, Wanda Maglione, Antonella Riccardi, Francesca Pignataro, Roberta Ferrara, Domenico Sambataro, Gianluca Sambataro, Claudio Vitali, Gabriele Valentini, Nicoletta Del Papa
BACKGROUND: Some abnormalities in nailfold videocapillaroscopy (NVC), such as the presence of micro-haemorrhages (MHEs), micro-thromboses (MTs), giant capillaries (GCs) and reduction in the number of capillaries (nCs), suggest a disease activity (DA) phase in systemic sclerosis (SSc). In a previous paper, we showed that the number of micro-haemorrhages and micro-thromboses (the so-called NEMO score) was the NVC feature more closely associated with DA. The present study was aimed at validating the NEMO score as a measure of DA in patients with SSc...
June 13, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28607106/ubash3a-mediates-risk-for-type-1-diabetes-through-inhibition-of-t-cell-receptor-induced-nf-%C3%AE%C2%BAb-signaling
#5
Yan Ge, Taylor K Paisie, Jeremy R B Newman, Lauren M McIntyre, Patrick Concannon
Although over 40 type 1 diabetes (T1D) risk loci have been mapped in humans, the causative genes and variants for T1D are largely unknown. Here, we investigated a candidate gene in the 21q22.3 risk locus-UBASH3A, which is primarily expressed in T cells where it is thought to play a largely redundant role. Genetic variants in UBASH3A have been shown to be associated with several autoimmune diseases in addition to T1D. However, the molecular mechanism underlying these genetic associations is unresolved. Our study reveals a previously unrecognized role of UBASH3A in human T cells: UBASH3A attenuates the NF-κB signal transduction upon T cell receptor (TCR) stimulation by specifically suppressing the activation of the IKK complex...
June 12, 2017: Diabetes
https://www.readbyqxmd.com/read/28572277/immunotherapies-in-neuromyelitis-optica-spectrum-disorder-efficacy-and-predictors-of-response
#6
Jan-Patrick Stellmann, Markus Krumbholz, Tim Friede, Anna Gahlen, Nadja Borisow, Katrin Fischer, Kerstin Hellwig, Florence Pache, Klemens Ruprecht, Joachim Havla, Tania Kümpfel, Orhan Aktas, Hans-Peter Hartung, Marius Ringelstein, Christian Geis, Christoph Kleinschnitz, Achim Berthele, Bernhard Hemmer, Klemens Angstwurm, Kim Lea Young, Simon Schuster, Martin Stangel, Florian Lauda, Hayrettin Tumani, Christoph Mayer, Lena Zeltner, Ulf Ziemann, Ralf Andreas Linker, Matthias Schwab, Martin Marziniak, Florian Then Bergh, Ulrich Hofstadt-van Oy, Oliver Neuhaus, Uwe Zettl, Jürgen Faiss, Brigitte Wildemann, Friedemann Paul, Sven Jarius, Corinna Trebst, Ingo Kleiter
OBJECTIVE: To analyse predictors for relapses and number of attacks under different immunotherapies in patients with neuromyelitis optica spectrum disorder (NMOSD). DESIGN: This is a retrospective cohort study conducted in neurology departments at 21 regional and university hospitals in Germany. Eligible participants were patients with aquaporin-4-antibody-positive or aquaporin-4-antibody-negative NMOSD. Main outcome measures were HRs from Cox proportional hazard regression models adjusted for centre effects, important prognostic factors and repeated treatment episodes...
June 1, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28559054/continuous-monitoring-of-noise-levels-in-the-gulf-of-catania-ionian-sea-study-of-correlation-with-ship-traffic
#7
S Viola, R Grammauta, V Sciacca, G Bellia, L Beranzoli, G Buscaino, F Caruso, F Chierici, G Cuttone, A D'Amico, V De Luca, D Embriaco, P Favali, G Giovanetti, G Marinaro, S Mazzola, F Filiciotto, G Pavan, C Pellegrino, S Pulvirenti, F Simeone, F Speziale, G Riccobene
Acoustic noise levels were measured in the Gulf of Catania (Ionian Sea) from July 2012 to May 2013 by a low frequency (<1000Hz) hydrophone, installed on board the NEMO-SN1 multidisciplinary observatory. NEMO-SN1 is a cabled node of EMSO-ERIC, which was deployed at a water depth of 2100m, 25km off Catania. The study area is characterized by the proximity of mid-size harbors and shipping lanes. Measured noise levels were correlated with the passage of ships tracked with a dedicated AIS antenna. Noise power was measured in the frequency range between 10Hz and 1000Hz...
May 28, 2017: Marine Pollution Bulletin
https://www.readbyqxmd.com/read/28543390/the-effect-of-ebv-on-wif1-nlk-and-apc-gene-methylation-and-expression-in-gastric-carcinoma-and-nasopharyngeal-cancer
#8
Zhenzhen Zhao, Wen Liu, Jincheng Liu, Jiayi Wang, Bing Luo
Epstein-Barr virus (EBV) is an important DNA tumor virus that is associated with approximately 10% of gastric carcinomas and 99% of nasopharyngeal cancers. DNA methylation and microRNAs (miRNAs) are the most studied epigenetic mechanisms that can prompt disease susceptibility. This study aimed to detect the effect of EBV on Wnt inhibitory factor 1 (WIF1), Nemo-like kinase (NLK), and adenomatous polyposis coli (APC) gene methylation and expression in gastric carcinoma and nasopharyngeal cancer. METHODS: The WIF1, NLK, and APC gene mRNA expression levels were measured by real-time quantitative RT-PCR in four EBV-positive cell lines and four EBV-negative cell lines...
May 23, 2017: Journal of Medical Virology
https://www.readbyqxmd.com/read/28521627/disseminated-bacillus-calmette-gu%C3%A3-rin-osteomyelitis-in-twin-sisters-related-to-stat1-gene-deficiency
#9
Sabah Boudjemaa, Linda Dainese, Sébastien Héritier, Caroline Masserot, Samia Hachemane, Jean-Laurent Casanova, Aurore Coulomb, Jacinta Bustamante
Mendelian susceptibility to mycobacterial disease is a rare syndrome characterized by severe clinical infections usually caused by weakly virulent mycobacterial species such as Bacillus Calmette-Guérin vaccines and environmental nontuberculous mycobacteria or more virulent mycobacteria as mycobacterium tuberculosis. Since 1996, 9 genes including 7 autosomal ( STAT1, IFNGR1, IFNGR2, IL12B, IL12RB1, ISG15, and IRF8) and 2 X-linked genes ( NEMO and CYBB) have been identified. Allelic heterogeneity leaded to recognize about 18 genetic diseases with variable clinical phenotypes, but sharing a same physiological mechanism represented by a defect in human IL-12-dependant-INF-γ-mediated immunity...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28518071/nerve-sparing-mid-urethral-obstruction-nemo-in-female-small-rodents
#10
Martin Sidler, Karen J Aitken, Jia Xin Jiang, Darius J Bägli
Partial bladder outlet obstruction (pBOO) has a high prevalence, causes significant patient burden, and immense health care costs. The most common animal model to investigate bladder remodeling in pBOO are female rodents undergoing partial obstruction at the proximal urethra. Variability in the degree of obstruction and animal mortality are major concerns with proximal obstruction. Furthermore, dissecting around the proximal urethra and bladder neck jeopardizes bladder innervation. We developed a nerve-sparing mid-urethral obstruction (NeMO) model for pBOO avoiding the disadvantages of the traditional model...
April 25, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28515292/molluscum-contagiosum-virus-mc159-abrogates-ciap1-nemo-interactions-and-inhibits-nemo-polyubiquitination
#11
Sunetra Biswas, Joanna L Shisler
Molluscum contagiosum virus (MCV) is a dermatotropic poxvirus that causes benign skin lesions. MCV lesions persist because of virally-encoded immune evasion molecules that inhibit anti-viral responses. The MCV MC159 protein suppresses NF-κB activation, a powerful anti-viral response, via interactions with the NEMO subunit of the IKK complex. Binding of MC159 to NEMO does not disrupt the IKK complex, implying that MC159 prevents IKK activation via an as-yet-identified strategy. Here, we demonstrated that MC159 inhibited NEMO polyubiquitination, a post-translational modification required for IKK and downstream NF-κB activation...
May 17, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28515148/nemo-a-transcriptional-target-of-estrogen-and-progesterone-is-linked-to-tumor-suppressor-pml-in-breast-cancer
#12
Kelli E Valdez, Hanan S Elsarraj, Yan Hong, Sandra L Grimm, Lawrence R Ricci, Fang Fan, Ossama Tawfik, Lisa May, Therese Cusick, Marc Inciardi, Mark Redick, Jason Gatewood, Onalisa Winblad, Susan G Hilsenbeck, Dean P Edwards, Christy Hagan, Andrew K Godwin, Carol J Fabian, Fariba Behbod
The beneficial versus detrimental roles of estrogen plus progesterone (E+P) in breast cancer remains controversial. Here we report a beneficial mechanism of E+P treatment in breast cancer cells driven by transcriptional upregulation of the NFκB modulator NEMO, which in turn promotes expression of the tumor suppressor protein PML. E+P treatment of patient-derived epithelial cells derived from ductal carcinoma in situ (DCIS) increased secretion of the pro-inflammatory cytokine IL-6. Mechanistic investigations indicated that IL-6 upregulation occurred as a result of transcriptional upregulation of NEMO, the gene for which harbored estrogen receptor (ER) binding sites within its promoter...
May 17, 2017: Cancer Research
https://www.readbyqxmd.com/read/28513998/ibd-due-to-pid-inflammatory-bowel-disease-caused-by-primary-immunodeficiencies-clinical-presentations-review-of-literature-and-proposal-of-a-rational-diagnostic-algorithm
#13
REVIEW
Daniel Tegtmeyer, Maximilian Seidl, Patrick Gerner, Ulrich Baumann, Christian Klemann
Inflammatory bowel diseases (IBD) including Crohn's disease (CD) and ulcerative colitis (UC) have a multifactorial pathogenesis with complex interactions between polygenetic predispositions and environmental factors. However, IBD can also be caused by monogenic diseases, such as primary immunodeficiencies (PID). Recently, an increasing number of these altogether rare diseases has been described to present often primarily, or solely as IBD. Early recognition of these conditions enables adaption of therapies and thus directly benefits the course of IBDs...
May 17, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28512628/dysplasia-of-granulocytes-in-a-patient-with-hpv-disease-recurrent-infections-and-b-lymphopenia-a-novel-variant-of-whim-syndrome
#14
Giusella M F Moscato, Erica Giacobbi, Lucia Anemona, Silvia Di Cesare, Gigliola Di Matteo, Massimo Andreoni, Alessandro Mauriello, Viviana Moschese
WHIM syndrome is a condition in which affected persons have chronic peripheral neutropenia, lymphopenia, abnormal susceptibility to human papilloma virus infection, and myelokathexis. Myelokathexis refers to the retention of mature neutrophils in the bone marrow (BM), which accounts for degenerative changes and hypersegmentation. Most patients present heterozygous autosomal dominant mutations of the gene encoding CXCR4. Consequently, aberrant CXCL12/CXCR4 signaling impairs the receptor downregulation causing hyperactivation (gain-of-function) that affects BM homing for myelopoiesis and lymphopoiesis and the release of neutrophils in the bloodstream...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28502111/asap3-upregulation-contributes-to-colorectal-carcinogenesis-and-indicates-poor-survival-outcome
#15
Haiying Tian, Jin Qian, Luoyan Ai, Yueyuan Li, Wenyu Su, Xian-Ming Kong, Jie Xu, Jing-Yuan Fang
The function and clinical implication of ArfGAP With SH3 Domain, Ankyrin Repeat And PH Domain 3 (ASAP3) in colorectal cancer (CRC) remains undefined. In the present study, we showed that the expression level of ASAP3 was dramatically increased in CRC and its upregulation was associated with American Joint Committee on Cancer (AJCC) stage (P<0.001) and poor prognosis (P=0.0022). The combination of AJCC stage and ASAP3 expression improved the prediction of survival in CRC patients. Suppression of ASAP3 inhibited cell proliferation by inducing G1-phase arrest without influencing apoptosis...
May 14, 2017: Cancer Science
https://www.readbyqxmd.com/read/28501618/assessing-activity-of-hepatitis-a-virus-3c-protease-using-a-cyclized-luciferase-based-biosensor
#16
Junwei Zhou, Dang Wang, Yongqiang Xi, Xinyu Zhu, Yuting Yang, Mengting Lv, Chuanzhen Luo, Jiyao Chen, Xu Ye, Liurong Fang, Shaobo Xiao
Hepatitis A is an acute infection caused by Hepatitis A virus (HAV), which is widely distributed throughout the world. The HAV 3C cysteine protease (3C(pro)), an important nonstructural protein, is responsible for most cleavage within the viral polyprotein and is critical for the processes of viral replication. Our group has previously demonstrated that HAV 3C(pro) cleaves human NF-κB essential modulator (NEMO), a kinase required in interferon signaling. Based on this finding, we generated four luciferase-based biosensors containing the NEMO sequence (PVLKAQ↓ADIYKA) that is cleaved by HAV 3C(pro) and/or the Nostoc punctiforme DnaE intein, to monitor the activity of HAV 3C(pro) in human embryonic kidney cells (HEK-293T)...
May 10, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28490597/molluscum-contagiosum-virus-protein-mc005-inhibits-nf%C3%AE%C2%BAb-activation-by-targeting-nemo-regulated-ikk-activation
#17
Gareth Brady, Darya A Haas, Paul J Farrell, Andreas Pichlmair, Andrew G Bowie
Molluscum Contagiosum Virus (MCV), the only known extant, human-adapted poxvirus, causes a long-duration infection characterized by skin lesions that typically display an absence of inflammation despite containing high titres of live virus. Despite this curious presentation, MCV is very poorly characterized in terms of host-pathogen interactions. The absence of inflammation around MCV lesions suggests the presence of potent inhibitors of human anti-viral immunity and inflammation. However, only a small number of MCV immunomodulatory genes have been characterized in detail...
May 10, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28481361/linear-ubiquitination-of-cytosolic-salmonella-typhimurium-activates-nf-%C3%AE%C2%BAb-and-restricts-bacterial-proliferation
#18
Sjoerd J L van Wijk, Franziska Fricke, Lina Herhaus, Jalaj Gupta, Katharina Hötte, Francesco Pampaloni, Paolo Grumati, Manuel Kaulich, Yu-Shin Sou, Masaaki Komatsu, Florian R Greten, Simone Fulda, Mike Heilemann, Ivan Dikic
Ubiquitination of invading Salmonella Typhimurium triggers autophagy of cytosolic bacteria and restricts their spread in epithelial cells. Ubiquitin (Ub) chains recruit autophagy receptors such as p62/SQSTM1, NDP52/CALCOCO and optineurin (OPTN), which initiate the formation of double-membrane autophagosomal structures and lysosomal destruction in a process known as xenophagy. Besides this, the functional consequences and mechanistic regulation of differentially linked Ub chains at the host-Salmonella interface have remained unexplored...
May 8, 2017: Nature Microbiology
https://www.readbyqxmd.com/read/28481331/lubac-synthesized-linear-ubiquitin-chains-restrict-cytosol-invading-bacteria-by-activating-autophagy-and-nf-%C3%AE%C2%BAb
#19
Jessica Noad, Alexander von der Malsburg, Claudio Pathe, Martin A Michel, David Komander, Felix Randow
Cell-autonomous immunity relies on the ubiquitin coat surrounding cytosol-invading bacteria functioning as an 'eat-me' signal for xenophagy. The origin, composition and precise mode of action of the ubiquitin coat remain incompletely understood. Here, by studying Salmonella Typhimurium, we show that the E3 ligase LUBAC generates linear (M1-linked) polyubiquitin patches in the ubiquitin coat, which serve as antibacterial and pro-inflammatory signalling platforms. LUBAC is recruited via its subunit HOIP to bacterial surfaces that are no longer shielded by host membranes and are already displaying ubiquitin, suggesting that LUBAC amplifies and refashions the ubiquitin coat...
May 8, 2017: Nature Microbiology
https://www.readbyqxmd.com/read/28479328/clinical-characteristics-of-patients-with-low-functional-il-6-production-upon-tlr-il-1r-stimulation
#20
Glynis Frans, Jutte Van der Werff Ten Bosch, Leen Moens, Greet Wuyts, Heidi Schaballie, David Tuerlinckx, Mia De Bie, Francois Vermeulen, Rik Schrijvers, Wim Meert, Matthew S Hestand, Joris Delanghe, Joris R Vermeesch, Isabelle Meyts, Xavier Bossuyt
No abstract text is available yet for this article.
May 5, 2017: Journal of Allergy and Clinical Immunology
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