Prevalence of the C282Y and H63D mutations of the HFE hemochromatosis gene in Azerian major β-thalassemia and iron overload

In this study, we have determined the allele frequency of HFE mutations H63D and C282Y in a group of Azerian beta-thalassemia major patients. These two mutations are implicated in hereditary hemochromatosis among Caucasians. In this study, we wanted to outbreak these mutations with the iron status in major beta-thalassemia patients. Sixty Azerian major beta-thalassemias were screening for the C282Y and H63D by digestion of polymerase chain reaction products (PCRP). Serum ferritin level was measured by enzyme-linked immunosorbent assay (ELISA). The allele frequency of H63D mutation was 20 . C282Y mutation was not present in our studied patients. No statistically significant difference of serum ferritin level was found between major beta-thalassemia with and without HFE mutations. Our data suggest that H63D mutation is so frequent in Azerian major beta-thalassemia patients than in the general population and that the coinheritance of H63D mutation does not influence the severity of iron overload in these patients. © 2015, Springer-Verlag London

Status of the CMS magnet (MT17)

The CMS experiment (Compact Muon Solenoid) is a general-purpose detector designed to run at the highest luminosity at the CERN Large Hadron Collider (LHC). Its distinctive features include a 4 T superconducting solenoid with a free bore of 6 m diameter and 12.5-m length, enclosed inside a 10 000-ton return yoke. The magnet will be assembled and tested in a surface hall at Point 5 of the LHC at the beginning of 2004 before being transferred by heavy lifting means to an experimental hall 90 m below ground level. The design and construction of the magnet is a common project of the CMS Collaboration. The task is organized by a CERN based group with strong technical and contractual participation from CEA Saclay, ETH Zurich, Fermilab, INFN Genova, ITEP Moscow, University of Wisconsin and CERN. The magnet project will be described, with emphasis on the present status of the fabrication. (15 refs)

Performance of the CMS Cathode Strip Chambers with Cosmic Rays

The Cathode Strip Chambers (CSCs) constitute the primary muon tracking device in the CMS endcaps. Their performance has been evaluated using data taken during a cosmic ray run in fall 2008. Measured noise levels are low, with the number of noisy channels well below 1%. Coordinate resolution was measured for all types of chambers, and fall in the range 47 microns to 243 microns. The efficiencies for local charged track triggers, for hit and for segments reconstruction were measured, and are above 99%. The timing resolution per layer is approximately 5 ns

The Long-Baseline Neutrino Experiment: Exploring Fundamental Symmetries of the Universe

The preponderance of matter over antimatter in the early Universe, the dynamics of the supernova bursts that produced the heavy elements necessary for life and whether protons eventually decay --- these mysteries at the forefront of particle physics and astrophysics are key to understanding the early evolution of our Universe, its current state and its eventual fate. The Long-Baseline Neutrino Experiment (LBNE) represents an extensively developed plan for a world-class experiment dedicated to addressing these questions. LBNE is conceived around three central components: (1) a new, high-intensity neutrino source generated from a megawatt-class proton accelerator at Fermi National Accelerator Laboratory, (2) a near neutrino detector just downstream of the source, and (3) a massive liquid argon time-projection chamber deployed as a far detector deep underground at the Sanford Underground Research Facility. This facility, located at the site of the former Homestake Mine in Lead, South Dakota, is approximately 1,300 km from the neutrino source at Fermilab -- a distance (baseline) that delivers optimal sensitivity to neutrino charge-parity symmetry violation and mass ordering effects. This ambitious yet cost-effective design incorporates scalability and flexibility and can accommodate a variety of upgrades and contributions. With its exceptional combination of experimental configuration, technical capabilities, and potential for transformative discoveries, LBNE promises to be a vital facility for the field of particle physics worldwide, providing physicists from around the globe with opportunities to collaborate in a twenty to thirty year program of exciting science. In this document we provide a comprehensive overview of LBNE's scientific objectives, its place in the landscape of neutrino physics worldwide, the technologies it will incorporate and the capabilities it will possess.Comment: Major update of previous version. This is the reference document for LBNE science program and current status. Chapters 1, 3, and 9 provide a comprehensive overview of LBNE's scientific objectives, its place in the landscape of neutrino physics worldwide, the technologies it will incorporate and the capabilities it will possess. 288 pages, 116 figure

Performance and Operation of the CMS Electromagnetic Calorimeter

The operation and general performance of the CMS electromagnetic calorimeter using cosmic-ray muons are described. These muons were recorded after the closure of the CMS detector in late 2008. The calorimeter is made of lead tungstate crystals and the overall status of the 75848 channels corresponding to the barrel and endcap detectors is reported. The stability of crucial operational parameters, such as high voltage, temperature and electronic noise, is summarised and the performance of the light monitoring system is presented

Heparan sulfate proteoglycans: structure, protein interactions and cell signaling

Heparan sulfate proteoglycans are ubiquitously found at the cell surface and extracellular matrix in all the animal species. This review will focus on the structural characteristics of the heparan sulfate proteoglycans related to protein interactions leading to cell signaling. The heparan sulfate chains due to their vast structural diversity are able to bind and interact with a wide variety of proteins, such as growth factors, chemokines, morphogens, extracellular matrix components, enzymes, among others. There is a specificity directing the interactions of heparan sulfates and target proteins, regarding both the fine structure of the polysaccharide chain as well precise protein motifs. Heparan sulfates play a role in cellular signaling either as receptor or co-receptor for different ligands, and the activation of downstream pathways is related to phosphorylation of different cytosolic proteins either directly or involving cytoskeleton interactions leading to gene regulation. The role of the heparan sulfate proteoglycans in cellular signaling and endocytic uptake pathways is also discussed.Proteoglicanos de heparam sulfato são encontrados tanto superfície celular quanto na matriz extracelular em todas as espécies animais. Esta revisão tem enfoque nas características estruturais dos proteoglicanos de heparam sulfato e nas interações destes proteoglicanos com proteínas que levam à sinalização celular. As cadeias de heparam sulfato, devido a sua variedade estrutural, são capazes de se ligar e interagir com ampla gama de proteínas, como fatores de crescimento, quimiocinas, morfógenos, componentes da matriz extracelular, enzimas, entreoutros. Existe uma especificidade estrutural que direciona as interações dos heparam sulfatos e proteínas alvo. Esta especificidade está relacionada com a estrutura da cadeia do polissacarídeo e os motivos conservados da cadeia polipeptídica das proteínas envolvidas nesta interação. Os heparam sulfatos possuem papel na sinalização celular como receptores ou coreceptores para diferentes ligantes. Esta ligação dispara vias de sinalização celular levam à fosforilação de diversas proteínas citosólicas ou com ou sem interações diretas com o citoesqueleto, culminando na regulação gênica. O papel dos proteoglicanos de heparam sulfato na sinalização celular e vias de captação endocítica também são discutidas nesta revisão.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Universidade Federal de São Paulo (UNIFESP) Departamento de BioquímicaUniversidade Federal de São Paulo (UNIFESP) Departamento de OftalmologiaUNIFESP, Depto. de BioquímicaUNIFESP, Depto. de OftalmologiaSciEL

A transient simulation for a novel solar-geothermal cogeneration system with a selection of heat transfer fluids using thermodynamics analysis and ANN intelligent (AI) modeling

Today, due to the reduction of fossil resources and on the other hand the high environmental pollution of these resources, it is necessary to pay attention to alternative resources, including clean and available renewable resources. In this study, solar and geothermal energy sources are combined, generating electricity, cooling, freshwater, and hydrogen, along with selecting a suitable heat transfer fluid for parabolic trough solar collectors. This system consists of parabolic trough solar collectors, a steam Rankine cycle, a steam Rankine cycle with an organic Rankine cycle, a proton exchange membrane electrolyzer, and a reverse osmosis desalination unit. To analyze the performance of solar collectors, Therminol 59, Marlotherm SH, Syltherm 800, and Therminol VP1 heat transfer fluids are selected. Solar radiation intensity, solar collector mass flow rate, turbine and pump efficiency, evaporator pinch-point temperature, and organic Rankine cycle turbine inlet temperature were investigated as design parameters affecting system performance. Maximizing energy efficiency and reducing cost rate were selected as two objective functions and determined using a multi-objective sorting genetic algorithm (NSGA-II). The results showed that Therminol 59 has the highest energy efficiency and net power output compared to other heat transfer fluids. Also, the proposed system produces 1140 kW of electricity in the optimal state, and in the best state, it has an energy efficiency of 32.39% and a cost of 36.32 $/GJ

The Long-Baseline Neutrino Experiment: Exploring Fundamental Symmetries of the Universe

Major update of previous version. This is the reference document for LBNE science program and current status. Chapters 1, 3, and 9 provide a comprehensive overview of LBNE's scientific objectives, its place in the landscape of neutrino physics worldwide, the technologies it will incorporate and the capabilities it will possess. 288 pages, 116 figuresMajor update of previous version. This is the reference document for LBNE science program and current status. Chapters 1, 3, and 9 provide a comprehensive overview of LBNE's scientific objectives, its place in the landscape of neutrino physics worldwide, the technologies it will incorporate and the capabilities it will possess. 288 pages, 116 figuresThe preponderance of matter over antimatter in the early Universe, the dynamics of the supernova bursts that produced the heavy elements necessary for life and whether protons eventually decay --- these mysteries at the forefront of particle physics and astrophysics are key to understanding the early evolution of our Universe, its current state and its eventual fate. The Long-Baseline Neutrino Experiment (LBNE) represents an extensively developed plan for a world-class experiment dedicated to addressing these questions. LBNE is conceived around three central components: (1) a new, high-intensity neutrino source generated from a megawatt-class proton accelerator at Fermi National Accelerator Laboratory, (2) a near neutrino detector just downstream of the source, and (3) a massive liquid argon time-projection chamber deployed as a far detector deep underground at the Sanford Underground Research Facility. This facility, located at the site of the former Homestake Mine in Lead, South Dakota, is approximately 1,300 km from the neutrino source at Fermilab -- a distance (baseline) that delivers optimal sensitivity to neutrino charge-parity symmetry violation and mass ordering effects. This ambitious yet cost-effective design incorporates scalability and flexibility and can accommodate a variety of upgrades and contributions. With its exceptional combination of experimental configuration, technical capabilities, and potential for transformative discoveries, LBNE promises to be a vital facility for the field of particle physics worldwide, providing physicists from around the globe with opportunities to collaborate in a twenty to thirty year program of exciting science. In this document we provide a comprehensive overview of LBNE's scientific objectives, its place in the landscape of neutrino physics worldwide, the technologies it will incorporate and the capabilities it will possess

The 2010 Interim Report of the Long-Baseline Neutrino Experiment Collaboration Physics Working Groups

Corresponding author R.J.Wilson ([email protected]); 113 pages, 90 figuresCorresponding author R.J.Wilson ([email protected]); 113 pages, 90 figuresIn early 2010, the Long-Baseline Neutrino Experiment (LBNE) science collaboration initiated a study to investigate the physics potential of the experiment with a broad set of different beam, near- and far-detector configurations. Nine initial topics were identified as scientific areas that motivate construction of a long-baseline neutrino experiment with a very large far detector. We summarize the scientific justification for each topic and the estimated performance for a set of far detector reference configurations. We report also on a study of optimized beam parameters and the physics capability of proposed Near Detector configurations. This document was presented to the collaboration in fall 2010 and updated with minor modifications in early 2011

CMS physics technical design report : Addendum on high density QCD with heavy ions

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