Aliviaderos tipo laberinto y en tecla de piano

Las conferencias presentadas durante la Jornada Técnica sobre Avances en investigación aplicada en seguridad hidráulica de presas organizada por el Centro de Estudios Hidrográficos del CEDEX y la Universidad Politécnica de Madrid el mes de junio de 2013 versan sobre la mejora de la seguridad hidráulica de las presas, una materia de especial importancia en nuestro país (habida cuenta del elevado número de infraestructuras de regulación en servicio y de su antigüedad media) y constituye una tarea en la que tanto el CEDEX como la Universidad Politécnica de Madrid han trabajado activamente en los últimos años. El Departamento de presas de la ETSICCP, el Centro de Estudios Hidrográficos del CEDEX el Centro Internacional de Modelación Numérica (CIMNE), la empresa VEMSA y la compañía de ingeniería JESÚS GRANELL Ingenieros Consultores viene acometiendo un proyecto de investigación en materia de vertederos tipo laberinto. El objetivo principal de la investigación es la obtención de conocimientos y herramientas que permitan un diseño razonado de este tipo de estructuras hidráulicas. Además, se estudian otros efectos en su comportamiento, como son las condiciones hidráulicas de aproximación a la embocadura. La investigación se basa en el ensayo hidráulico en modelos físicos complementado por modelos numéricos mediante aplicaciones de última generación. Descripción del proceso de diseño y construcción del modelo

Identification of Rare LRP5 Variants in a Cohort of Males with Impaired Bone Mass

Osteoporosis is the most common bone disease characterized by reduced bone mass and increased bone fragility. Genetic contribution is one of the main causes of primary osteoporosis; therefore, both genders are affected by this skeletal disorder. Nonetheless, osteoporosis in men has received little attention, thus being underestimated and undertreated. The aim of this study was to identify novel genetic variants in a cohort of 128 males with idiopathic low bone mass using a next-generation sequencing (NGS) panel including genes whose mutations could result in reduced bone mineral density (BMD). Genetic analysis detected in eleven patients ten rare heterozygous variants within the LRP5 gene, which were categorized as VUS (variant of uncertain significance), likely pathogenic and benign variants according to American College of Medical Genetics and Genomics (ACMG) guidelines. Protein structural and Bayesian analysis performed on identified LRP5 variants pointed out p.R1036Q and p.R1135C as pathogenic, therefore suggesting the likely association of these two variants with the low bone mass phenotype. In conclusion, this study expands our understanding on the importance of a functional LRP5 protein in bone formation and highlights the necessity to sequence this gene in subjects with idiopathic low B

A novel DSP zebrafish model reveals training- and drug-induced modulation of arrhythmogenic cardiomyopathy phenotypes

Abstract Arrhythmogenic cardiomyopathy (AC) is an inherited disorder characterized by progressive loss of the ventricular myocardium causing life-threatening ventricular arrhythmias, syncope and sudden cardiac death in young and athletes. About 40% of AC cases carry one or more mutations in genes encoding for desmosomal proteins, including Desmoplakin (Dsp). We present here the first stable Dsp knock-out (KO) zebrafish line able to model cardiac alterations and cell signalling dysregulation, characteristic of the AC disease, on which environmental factors and candidate drugs can be tested. Our stable Dsp knock-out (KO) zebrafish line was characterized by cardiac alterations, oedema and bradycardia at larval stages. Histological analysis of mutated adult hearts showed reduced contractile structures and abnormal shape of the ventricle, with thinning of the myocardial layer, vessels dilation and presence of adipocytes within the myocardium. Moreover, TEM analysis revealed “pale”, disorganized and delocalized desmosomes. Intensive physical training protocol caused a global worsening of the cardiac phenotype, accelerating the progression of the disease. Of note, we detected a decrease of Wnt/β-catenin signalling, recently associated with AC pathogenesis, as well as Hippo/YAP-TAZ and TGF-β pathway dysregulation. Pharmacological treatment of mutated larvae with SB216763, a Wnt/β-catenin agonist, rescued pathway expression and cardiac abnormalities, stabilizing the heart rhythm. Overall, our Dsp KO zebrafish line recapitulates many AC features observed in human patients, pointing at zebrafish as a suitable system for in vivo analysis of environmental modulators, such as the physical exercise, and the screening of pathway-targeted drugs, especially related to the Wnt/β-catenin signalling cascade

Neandertales y Humanos modernos en Guadalajara

Con autorización de la revista para autores CSIC[EN] We present partial results obtained in an interdisciplinary research project focused on the human settlement of the Guadalajara province (Spain) during the Middle and Upper Palaeolithic. The excavation of the Peña Capón, Peña Cabra and Los Casares sites have shown outstanding evidence for investigating population dynamics and human-environment interactions in the interior territories of the Iberian Peninsula during the Late Pleistocene. Traditionally depicted as marginal and lacking own cultural developments, these territories have provided scarce and weak data for the Middle and –especially– Upper Paleolithic, and thus the proposed interpretations on the mentioned problems have been always flawed. However, our results enable us to confirm the cultural relevance of the region under study during Upper Pleniglacial times previously considered devoid of human occupation. Also, we are now able to contribute with solid data from inland Spain to the problem of the Neandertal demise in the Iberian Peninsula and southwest Europe.[ES] Presentamos parte de los resultados de un proyecto de investigación interdisciplinar sobre el poblamiento humano en la provincia de Guadalajara durante el Paleolítico Medio y el Superior. Basados en la excavación de los yacimientos de Peña Capón, Peña Cabra y Los Casares, los datos obtenidos presentan importantes implicaciones para la investigación de las dinámicas poblaciones y la relación entre ocupación humana y variabilidad climática y ambiental en el interior de la península ibérica durante el Pleistoceno Superior. Tradicionalmente consideradas marginales, las regiones interiores peninsulares han aportado escasas evidencias relevantes para estas cronologías, provocando así que las interpretaciones sobre los problemas aludidos sean altamente cuestionables. Sin embargo, los resultados obtenidos nos permiten afirmar la entidad cultural de la región estudiada durante momentos del Pleniglacial Superior antes considerados deshabitados, así como contribuir con evidencias sólidas a la controversia del final de los Neandertales en la península ibérica.Gran parte de las investigaciones han sido financiadas a través de un proyecto Marie Curie (IEF) dentro del Séptimo Programa Marco (FP7) de la Comisión Europea (Identificador: 628179) así como por la Fundación Alemana de Investigación (DFG) bajo el CRC 806 “Our Way to Europe”. También se ha contado con financiación proveniente de los siguientes proyectos del Ministerio de Economía y Competitividad financiados por la Agencia Estatal de Investigación (AEI) y el Fondo Europeo de Desarrollo Regional (FEDER): HAR2013-48784-C3-3-P, HAR2016-76760-C3-2-P, HAR2013-43701-P, CGL2012-38434-C03-01 y HAR2017-82483-C3-3-P. Manuel Alcaraz-Castaño disfruta de un contrato co-financiado por las Ayudas para la Atracción de Talento Investigador de la Comunidad de Madrid (referencia 2016-T2/HUM-1251)

Tea Bag Index S and k data of tidal wetland sites, within-site high vs. low

Coverage: EVENT LABEL: Ameland * LATITUDE: 53.400000 * LONGITUDE: 5.800000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Coon_Island * LATITUDE: 38.200000 * LONGITUDE: -122.470000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Dieksanderkoog * LATITUDE: 54.000000 * LONGITUDE: 8.890000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Dipper_Harbour * LATITUDE: 45.100000 * LONGITUDE: -66.430000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Dongtan * LATITUDE: 31.500000 * LONGITUDE: 121.960000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Isla_Cristobal * LATITUDE: 9.400000 * LONGITUDE: -82.240000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Isla_Popa * LATITUDE: 9.400000 * LONGITUDE: -82.120000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Isla_Solarte * LATITUDE: 9.400000 * LONGITUDE: -82.200000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Laws_Point * LATITUDE: 42.700000 * LONGITUDE: -70.840000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Noord-Friesland_Buitendijks * LATITUDE: 53.400000 * LONGITUDE: 5.770000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Patuxent_River * LATITUDE: 38.700000 * LONGITUDE: -76.710000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Rhode_River * LATITUDE: 38.900000 * LONGITUDE: -76.550000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Rimouski * LATITUDE: 48.100000 * LONGITUDE: -69.790000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Rush_Ranch * LATITUDE: 38.200000 * LONGITUDE: -122.030000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Sonke-Nissen-Koog * LATITUDE: 54.600000 * LONGITUDE: 8.840000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Spiekeroog * LATITUDE: 53.800000 * LONGITUDE: 7.720000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: TIDE_project * LATITUDE: 42.700000 * LONGITUDE: -70.830000 * METHOD/DEVICE: Multiple investigations EVENT LABEL: Twin_Cays * LATITUDE: 16.833300 * LONGITUDE: -88.100000 * LOCATION: Caribbean Sea * METHOD/DEVICE: Multiple investigations EVENT LABEL: Venice_Lagoon * LATITUDE: 45.450000 * LONGITUDE: 12.310000 * LOCATION: Venice, Italy * METHOD/DEVICE: Multiple investigations EVENT LABEL: Wachapreague * LATITUDE: 37.580000 * LONGITUDE: -75.650000 * LOCATION: North Atlantic Ocea

Measurement of the Ωc0\Omega_c^0 lifetime at Belle II

We report on a measurement of the $\Omega_c^0$ lifetime using $\Omega_c^0 \to \Omega^-\pi^+$ decays reconstructed in $e^+e^-\to c\bar{c}$ data collected by the Belle II experiment and corresponding to $207~{\rm fb^{-1}}$ of integrated luminosity. The result, $\rm\tau(\Omega_c^0)=243\pm48( stat)\pm11(syst)~fs$, agrees with recent measurements indicating that the $\Omega_c^0$ is not the shortest-lived weakly decaying charmed baryon

Measurement of the Ωc0\Omega_c^0 lifetime at Belle II

We report on a measurement of the $\Omega_c^0$ lifetime using $\Omega_c^0 \to \Omega^-\pi^+$ decays reconstructed in $e^+e^-\to c\bar{c}$ data collected by the Belle II experiment and corresponding to $207~{\rm fb^{-1}}$ of integrated luminosity. The result, $\rm\tau(\Omega_c^0)=243\pm48( stat)\pm11(syst)~fs$, agrees with recent measurements indicating that the $\Omega_c^0$ is not the shortest-lived weakly decaying charmed baryon

Measurement of the Ωc0\Omega_c^0 lifetime at Belle II

We report on a measurement of the $\Omega_c^0$ lifetime using $\Omega_c^0 \to \Omega^-\pi^+$ decays reconstructed in $e^+e^-\to c\bar{c}$ data collected by the Belle II experiment and corresponding to $207~{\rm fb^{-1}}$ of integrated luminosity. The result, $\rm\tau(\Omega_c^0)=243\pm48( stat)\pm11(syst)~fs$, agrees with recent measurements indicating that the $\Omega_c^0$ is not the shortest-lived weakly decaying charmed baryon