The materials production and processing facility at the Spanish National Centre for fusion technologies (TechnoFusión)

Proceedings of : The 26th Symposium of Fusion Technology (SOFT-26), Porto (Portugal),- 27 september - 1 october 2010.In response to the urgent request from the EU Fusion Program, a new facility (TechnoFusion) for research and development of fusion materials has been planned with support from the Regional Government of Madrid and the Ministry of Science and Innovation of Spain. TechnoFusion, the National Centre for Fusion Technologies, aims screening different technologies relevant for ITER and DEMO environments while promoting the contribution of international companies and research groups into the Fusion Programme. For this purpose, the centre will be provided with a large number of unique facilities for the manufacture, testing (a triple-beam multi-ion irradiation, a plasma-wall interaction device, a remote handling for under ionizing radiation testing) and analysis of critical fusion materials. Particularly, the objectives, semi-industrial scale capabilities and present status of the TechnoFusion Materials Production and Processing (MPP) facility are presented. Previous studies revealed that the MPP facility will be a very promising infrastructure for the development of new materials and prototypes demanded by the fusion technology and therefore some of them will be here briefly summarized.This work has been supported by Madrid Community through the project TECHNOFUSION (S2009/ENE/1679).Publicad

Structural and doping effects in the half-metallic double perovskite A2A_2CrWO6_6

he structural, transport, magnetic and optical properties of the double perovskite $A_2$CrWO$_6$ with $A=\text{Sr, Ba, Ca}$ have been studied. By varying the alkaline earth ion on the $A$ site, the influence of steric effects on the Curie temperature $T_C$ and the saturation magnetization has been determined. A maximum $T_C=458$ K was found for Sr$_2$CrWO$_6$ having an almost undistorted perovskite structure with a tolerance factor $f\simeq 1$. For Ca$_2$CrWO$_6$ and Ba$_2$CrWO$_6$ structural changes result in a strong reduction of $T_C$. Our study strongly suggests that for the double perovskites in general an optimum $T_C$ is achieved only for $f \simeq 1$, that is, for an undistorted perovskite structure. Electron doping in Sr$_2$CrWO$_6$ by a partial substitution of Sr$^{2+}$ by La$^{3+}$ was found to reduce both $T_C$ and the saturation magnetization $M_s$. The reduction of $M_s$ could be attributed both to band structure effects and the Cr/W antisites induced by doping. Band structure calculations for Sr$_2$CrWO$_6$ predict an energy gap in the spin-up band, but a finite density of states for the spin-down band. The predictions of the band structure calculation are consistent with our optical measurements. Our experimental results support the presence of a kinetic energy driven mechanism in $A_2$CrWO$_6$, where ferromagnetism is stabilized by a hybridization of states of the nonmagnetic W-site positioned in between the high spin Cr-sites.Comment: 14 pages, 10 figure

Molecular profiling of immunoglobulin heavy-chain gene rearrangements unveils new potential prognostic markers for multiple myeloma patients

Altres ajuts: This work was partially supported by[...] , CIBERONC-CB16/12/00233, and "Una manera de hacer Europa" (Innocampus; CEI-2010-1-0010)". M.G.-A., I.P.-C., and C.J. are supported by the Fundación Española de Hematología y Hemoterapia (FEHH, co-funded by Fundación Cris in the latter case), A.M. by the European Social Fund and the Spanish Education Council through the University of Salamanca, [...]. All Spanish funding is co-sponsored by the European Union FEDER program.Multiple myeloma is a heterogeneous disease whose pathogenesis has not been completely elucidated. Although B-cell receptors play a crucial role in myeloma pathogenesis, the impact of clonal immunoglobulin heavy-chain features in the outcome has not been extensively explored. Here we present the characterization of complete heavy-chain gene rearrangements in 413 myeloma patients treated in Spanish trials, including 113 patients characterized by next-generation sequencing. Compared to the normal B-cell repertoire, gene selection was biased in myeloma, with significant overrepresentation of IGHV3, IGHD2 and IGHD3, as well as IGHJ4 gene groups. Hypermutation was high in our patients (median: 8.8%). Interestingly, regarding patients who are not candidates for transplantation, a high hypermutation rate (≥7%) and the use of IGHD2 and IGHD3 groups were associated with improved prognostic features and longer survival rates in the univariate analyses. Multivariate analysis revealed prolonged progression-free survival rates for patients using IGHD2/IGHD3 groups (HR: 0.552, 95% CI: 0.361−0.845, p = 0.006), as well as prolonged overall survival rates for patients with hypermutation ≥7% (HR: 0.291, 95% CI: 0.137−0.618, p = 0.001). Our results provide new insights into the molecular characterization of multiple myeloma, highlighting the need to evaluate some of these clonal rearrangement characteristics as new potential prognostic markers

Methodological Adaptation to EHEA of the of Instrumental Techniques subject of Pharmacy degree at the University of Barcelona

En el plan de estudios del Grado de Farmacia de la Universidad de Barcelona, la asignatura de Técnicas Instrumentales se imparte en el cuarto semestre, después de haber cursado Física, Fisicoquímica y Química Analítica. El equipo docente de la asignatura está integrado por once profesores que mediante trabajo colaborativo y adecuada coordinación organizan la docencia de la misma que se distribuye en clases teóricas y prácticas Con el objetivo de adaptar la asignatura a las necesidades del Espacio Europeo de Educación Superior, se distribuyó en tres Bloques: I, Técnicas Espectroscópicas; II, Técnicas Electroquímicas y III, Técnicas de Separación. Las actividades teórico-prácticas se han planificado de manera secuencial. Así se inicia el ciclo con las clases teóricas del Bloque I y a continuación de manera paralela se imparten las clases prácticas del Bloque I y las clases teóricas del Bloque II y así sucesivamente, de manera que se termina la docencia con las prácticas del último Bloque. En este proceso adquiere especial relevancia tanto la formación práctica en el laboratorio como el trabajo tutorizado que debe realizar el estudiante. Se realiza un proceso de evaluación continuada teórico/práctico en cada uno de los Bloques. Se da especial relevancia a la adquisición de habilidades y destrezas que permitan una correcta realización de las prácticas de laboratorio, es decir la integración de los contenidos específicos a la aplicación de las diferentes técnicas instrumentales, la resolución de los cálculos numéricos y la interpretación de los resultados.In the new syllabus of the Pharmacy degree at the University of Barcelona, the subject Analytical Techniques is taught at the fourth semester, after the subjects Physics, Physical chemistry and Analytical chemistry. The teaching team of this subject is integrated by eleven teachers that by means of collaborative work and an appropriate coordination, organize the docent activity into practical and theoretical classes. With the aim to adapt this subject to the requirements of the European space for higher education, it has been designed in three blocs: I. Spectroscopic techniques, II. Electrochemical techniques and, III. Separation techniques, by planning the theoretical and practical activities in a sequential manner. Therefore, the cycle begins with the theory of the first bloc followed with the practice corresponding to it together with the theory of the second bloc, and so on. The course ends with the practical part of the third bloc. In this process is of great importance the tutorial work that the student should do. The evaluation of the theory and of the practical part of each bloc is done in a continuous way paying special focus on the acquisition of abilities and handiness that will allow the correct performance in the laboratory. In summary, the integration of the specific contents to the application of the different instrumental techniques, the resolution of the numerical calculations and the interpretation of the results.Este trabajo ha estado subvencionado por los Proyectos: "Adaptació metodològical a l'Espai Europeu d'Educació Superior del projecte docent del Departament de Fisicoquímica per a la implementaió del nou grau de Farmàcia", nº 2009QMD00207. Programa "Millora de la qualitat docenta les universitats de Catalunya" - MQD2009 de la Generalitat de Catalunya". "Bones pràctiques d0avaluació continuada a la UB". Programa del 2009: Ajuts a projectes dìnnovació docent de la Universitat de Barcelon

PDGF-BB serum levels are decreased in adult onset Pompe patients

Adult onset Pompe disease is a genetic disorder characterized by slowly progressive skeletal and respiratory muscle weakness. Symptomatic patients are treated with enzymatic replacement therapy with human recombinant alfa glucosidase. Motor functional tests and spirometry are commonly used to follow patients up. However, a serological biomarker that correlates with the progression of the disease could improve follow-up. We studied serum concentrations of TGFβ, PDGF-BB, PDGF-AA and CTGF growth factors in 37 adult onset Pompe patients and 45 controls. Moreover, all patients performed several muscle function tests, conventional spirometry, and quantitative muscle MRI using 3-point Dixon. We observed a statistically significant change in the serum concentration of each growth factor in patients compared to controls. However, only PDGF-BB levels were able to differentiate between asymptomatic and symptomatic patients, suggesting its potential role in the follow-up of asymptomatic patients. Moreover, our results point to a dysregulation of muscle regeneration as an additional pathomechanism of Pompe disease

Estudio prospectivo, de seguimiento en pacientes con enfermedad de Gaucher Tipo 1 que reciben tratamiento con CERDELGA®. Proyecto TRAZELGA

Poster [PC-303] Introducción: La enfermedad de Gaucher tipo 1 (EG1), secundaria al déficit en la enzima glucocerebrosidasa lisosomal, provoca el acúmulo de glucocerebrósido principalmente en macrófagos, causando deterioro de los órganos en los que se deposita. El nuevo inhibidor de substrato Eliglustat (ELG), aprobado por la EMEA en 2015 y disponible desde enero 2017, inhibe de forma selectiva y potente la enzima glucosilceramida sintasa, disminuyendo el acúmulo de substrato, está indicado en EG1 metabolizadores rápidos, intermedios o lentos para el citocromo CYP2D6. Los ensayos clínicos de fase 2 y 3 demostraron mejora y estabilización de los parámetros tanto en los pacientes naïve, como en los de tratamiento enzimático sustitutivo. En este trabajo se expone el estudio de trazabilidad del tratamiento con eliglustat en pacientes con GD1 en España (TRAZELGA). Material y Métodos: El estudio nacional, multicéntrico TRAZELGA, ha sido diseñado como herramienta para evaluar de forma uniforme la respuesta al tratamiento durante un año, analizando los cambios en parámetros clínicos y biomarcadores habituales, registro de medicamentos concomitantes y efectos adversos a ELG, estudio de calidad de vida e incorporando un estudio exploratorio de marcadores de activación del sistema inmune (perfil de citoquinas, ferritina, lipocalina, gammaglobulinas, marcadores de estrés oxidativo), así como cambios en la infiltración medular cuantificados por RM y DEXA. Previo al inicio de ELG se realizó una evaluación de función cardíaca, hepática y renal. Resultados: 35 pacientes han iniciado tratamiento oral con Eliglustat. En esta presentación aportamos resultados preliminares de 21 pacientes (mediana de edad: 43, 8 años(23-75), 47% varones), genotipo de EG N370S/N370S: (29, 4%), N370S/L444P (41, 2%), otros dobles heterocigotos con N370S (29, 4%), metabolismo del CYP2D6 (12% metabolizadores lentos, 64, 5% intermedios y 33, 5% rápidos, ningún paciente recibió el tratamiento en prímera línea y sus características basales (tabla1), son de pacientes estabilizados con TES (15 casos) o miglustat (6). Un paciente esplenectomizado. 3 pacientes esplenomegalia palpable al momento de inclusión. 6 pacientes con multimorbilidades y polimedicaciones y 5 pacientes aquejaban astenia como síntoma principal antes de su inclusión en este estudio. El seguimiento medio actual es de 6 meses. Conclusiones: Se espera incluir un total de 30 pacientes en el estudio y analizar la influencia de Eliglustat sobre los biomarcadores, marcadores de inflamación, densidad mineral ósea. Tener información sobre adherencia, efectos adversos en práctica clínica habitual y grado de satisfacción. Aunque escasos, hasta ahora no hay publicada información de la respuesta al tratamiento en pacientes provenientes de tratamiento con miglustat. En caso de aceptación se presentará un análisis exhaustivo, invitando a todos los interesados a unirse al proyecto

Modifiable risk factors associated with prediabetes in men and women: A cross-sectional analysis of the cohort study in primary health care on the evolution of patients with prediabetes

Background: Prediabetes is a high-risk state for diabetes development, but little is known about the factors associated with this state. The aim of the study was to identify modifiable risk factors associated with the presence of prediabetes in men and women. Methods: Cohort Study in Primary Health Care on the Evolution of Patients with Prediabetes (PREDAPS-Study) is a prospective study on a cohort of 1184 subjects with prediabetes and another cohort of 838 subjects without glucose metabolism disorders. It is being conducted by 125 general practitioners in Spain. Data for this analysis were collected during the baseline stage in 2012. The modifiable risk factors included were: smoking habit, alcohol consumption, low physical activity, inadequate diet, hypertension, dyslipidemia, and obesity. To assess independent association between each factor and prediabetes, odds ratios (ORs) were estimated using logistic regression models. Results: Abdominal obesity, low plasma levels of high-density lipoprotein cholesterol (HDL-cholesterol), and hypertension were independently associated with the presence of prediabetes in both men and women. After adjusting for all factors, the respective ORs (95% Confidence Intervals) were 1.98 (1.41-2.79), 1.88 (1.23-2.88) and 1.86 (1.39-2.51) for men, and 1.89 (1.36-2.62), 1.58 (1.12-2.23) and 1.44 (1.07-1.92) for women. Also, general obesity was a risk factor in both sexes but did not reach statistical significance among men, after adjusting for all factors. Risky alcohol consumption was a risk factor for prediabetes in men, OR 1.49 (1.00-2.24). Conclusions: Obesity, low HDL-cholesterol levels, and hypertension were modifiable risk factors independently related to the presence of prediabetes in both sexes. The magnitudes of the associations were stronger for men than women. Abdominal obesity in both men and women displayed the strongest association with prediabetes. The findings suggest that there are some differences between men and women, which should be taken into account when implementing specific recommendations to prevent or delay the onset of diabetes in adult population

GWAS for Systemic Sclerosis Identifies Multiple Risk Loci and Highlights Fibrotic and Vasculopathy Pathways

Systemic sclerosis (SSc) is an autoimmune disease that shows one of the highest mortality rates among rheumatic diseases. We perform a large genome-wide association study (GWAS), and meta-analysis with previous GWASs, in 26,679 individuals and identify 27 independent genome-wide associated signals, including 13 new risk loci. The novel associations nearly double the number of genome-wide hits reported for SSc thus far. We define 95% credible sets of less than 5 likely causal variants in 12 loci. Additionally, we identify specific SSc subtype-associated signals. Functional analysis of high-priority variants shows the potential function of SSc signals, with the identification of 43 robust target genes through HiChIP. Our results point towards molecular pathways potentially involved in vasculopathy and fibrosis, two main hallmarks in SSc, and highlight the spectrum of critical cell types for the disease. This work supports a better understanding of the genetic basis of SSc and provides directions for future functional experiments.Funding: This work was supported by Spanish Ministry of Economy and Competitiveness (grant ref. SAF2015-66761-P), Consejeria de Innovacion, Ciencia y Tecnologia, Junta de Andalucía (P12-BIO-1395), Ministerio de Educación, Cultura y Deporte through the program FPU, Juan de la Cierva fellowship (FJCI-2015-24028), Red de Investigación en Inflamación y Enfermadades Reumaticas (RIER) from Instituto de Salud Carlos III (RD16/0012/0013), and Scleroderma Research Foundation and NIH P50-HG007735 (to H.Y.C.). H.Y.C. is an Investigator of the Howard Hughes Medical Institute. PopGen 2.0 is supported by a grant from the German Ministry for Education and Research (01EY1103). M.D.M and S.A. are supported by grant DoD W81XWH-18-1-0423 and DoD W81XWH-16-1-0296, respectively

The Mars Environmental Dynamics Analyzer, MEDA. A Suite of Environmental Sensors for the Mars 2020 Mission

86 pags., 49 figs., 24 tabs.NASA’s Mars 2020 (M2020) rover mission includes a suite of sensors to monitor current environmental conditions near the surface of Mars and to constrain bulk aerosol properties from changes in atmospheric radiation at the surface. The Mars Environmental Dynamics Analyzer (MEDA) consists of a set of meteorological sensors including wind sensor, a barometer, a relative humidity sensor, a set of 5 thermocouples to measure atmospheric temperature at ∼1.5 m and ∼0.5 m above the surface, a set of thermopiles to characterize the thermal IR brightness temperatures of the surface and the lower atmosphere. MEDA adds a radiation and dust sensor to monitor the optical atmospheric properties that can be used to infer bulk aerosol physical properties such as particle size distribution, non-sphericity, and concentration. The MEDA package and its scientific purpose are described in this document as well as how it responded to the calibration tests and how it helps prepare for the human exploration of Mars. A comparison is also presented to previous environmental monitoring payloads landed on Mars on the Viking, Pathfinder, Phoenix, MSL, and InSight spacecraft.This work has been funded by the Spanish Ministry of Economy and Competitiveness, through the projects No. ESP2014-54256-C4-1-R (also -2-R, -3-R and -4-R) and AYA2015-65041-P; Ministry of Science, Innovation and Universities, projects No. ESP2016-79612-C3-1-R (also -2-R and -3-R), ESP2016-80320-C2-1-R, RTI2018-098728-B-C31 (also -C32 and -C33) and RTI2018-099825-B-C31; Instituto Nacional de Técnica Aeroespacial; Ministry of Science and Innovation’s Centre for the Development of Industrial Technology; Grupos Gobierno Vasco IT1366-19; and European Research Council Consolidator Grant no 818602. The US co-authors performed their work under sponsorship from NASA’s Mars 2020 project, from the Game Changing Development program within the Space Technology Mission Directorate and from the Human Exploration and Operations Directorate