Análisis económico-financiero del Real Zaragoza S.A.D

Este trabajo tiene como objeto realizar un análisis de la situación económico-financiera de la empresa Real Zaragoza S.A.D durante el periodo 2009-2013 y su posterior comparativa con otros clubes de fútbol. Para ello se ha realizado un análisis patrimonial, de liquidez y solvencia y, de rentabilidad, con el que se ha posicionado a la empresa en el sector, desvelando con éste, la situación conflictiva en la que se ha encontrado el club a lo largo de los cinco años de estudio. Además, se muestra un análisis comparativo en el cual se ha realizado la media de los indicadores económico-financieros de 14 empresas del sector para poder realizar una conclusión acerca de qué medidas debería tomar dicho club para realizar actuaciones futuras que mejoren su gestión

Ecoetiques de serveis als establiments hotelers del Parc Natural de l'Alt Pirineu

En el present projecte s'analitza el turisme i la seva relació amb la figura del Parc Natural de l'Alt Pirineu (a partir d'ara PNAP). A la zona d'estudi el turisme és una de les principals activitats econòmiques, donat el notable valor ecologico-cultural de l'indret. L'activitat turística al PNAP és molt diversa, és per aquest motiu que s'observen diverses repercussions en l'entorn segons la tipologia de turisme que es doni. Donada aquesta realitat, l'estudi que es presenta està centrat en el sector hoteler de l'àmbit d'influència del PNAP. D'aquesta forma, s'analitza i diagnostica l'estat de qualitat ambiental dels hotels i pensions que componen el sector. Les eines emprades en aquest estudi han estat dues. Primerament, s'avalua la implantació del Distintiu de Garantia de Qualitat Ambiental (a partir d'ara DGQA). El DGQA és una ecoetiqueta de serveis, de recent creació, que atorga la Generalitat de Catalunya a partir de la revisió de l'establiment per mitjà d'un tècnic autoritzat. En segon terme, s'ha creat i emprat un producte anomenat Enquesta Bàsica d'Auto-xequeig de Qualitat Ambiental (a partir d'ara EBAQA). L'EBAQA té una doble finalitat. Per una banda respon a la necessitat d'autodiagnosi que tenen els propietaris, al ser aquests qui realitzen l'enquesta. Per altra banda, permet determinar quin és l'estat de qualitat ambiental del sector hoteler a la zona d'estudi a partir de l'avaluació de 35 dels 67 establiments existents a l'àmbit d'influència del PNAP, el qual representa una mostra del 61%.En el proyecto que se presenta se analiza el turismo y su relación con la figura del Parc Natural de l'Alt Pirineu (a partir de ahora PNAP). En la zona de estudio el turismo es una de las principales actividades económicas, dado el notable valor ecológico-cultural del lugar. La actividad turística en el PNAP es muy diversa, es por este motivo que se observan diversas repercusiones en el entorno según la tipología de turismo puesto en práctica. Dada esta realidad, el estudio que se presenta está centrado en el sector hotelero del ámbito de influencia del PNAP. De esta forma, se analiza y diagnostica el estado de calidad ambiental de los hoteles y pensiones que componen el sector. Han sido dos los instrumentos utilizados en este estudio. En primera instancia, se evalúa la implantación del Distintiu de Garantia de Qualitat Ambiental (a partir de ahora DGQA). El DGQA es una ecoetiqueta de servicios, de reciente creación, que otorga la Generalitat de Catalunya a partir de la revisión del establecimiento por un técnico autorizado. En segunda instancia, se ha creado y utilizado un producto llamado Enquesta Bàsica d'Auto-xequeig de Qualitat Ambiental (a partir de ahora EBAQA). La EBAQA tiene una doble finalidad. Por un lado, responde a la necesidad de autodiagnosis que tienen los propietarios, al ser estos quienes realizan la encuesta. Por otro lado, ésta permite determinar cual es el estado de calidad ambiental del sector hotelero de la zona de estudio, a partir de la evaluación de 35 de los 67 establecimientos existentes en el ámbito de influencia del PNAP, lo cual representa una muesstra del 61%.The current project analyzes tourism and its relationship with the protection's figure of the Parc Natural de l'Alt Pirineu (from now PNAP). In the studied area, tourism is a major economic activity, owing to ecological and cultural value of the parc. The tourism activity in the PNAP is very diverse; it is for this reason that there are various impacts on the environment according to the typology of the implemented tourism. Because of this reality, the study focuses on the hotel industry's sphere of influence of PNAP. Thus, the state of the environmental quality of hotels and guest houses is diagnosed. There have been two instruments used in this study. In the first instance, assesses the implementation of Distintiu de Garantia de Qualitat Ambiental (from now DGQA). The DGQA is an eco-label service, newly created, which is granted by the Generalitat de Catalunya from the review of the establishment by an authorized technician. In the second instance, a product called Enquesta Bàsica d'Auto-xequeig of Environmental Quality (from now EBAQA) was created and used. The EBAQA is twofold. On the first hand, responds to the need of having auto diagnosis owners, as these who performed the survey. On the other hand, it helps to determine which is the state of the environmental quality of the hotel sector of the study area, based on the evaluation of 35 out of 67 existing establishments in the area of influence of PNAP, which represents a sample of 61%

More than one HMG-CoA Lyase: The classical mitochondrial enzyme plus the peroxisomal and the cytosolic ones

There are three human enzymes with HMG-CoA lyase activity that are able to synthesize ketone bodies in different subcellular compartments. The mitochondrial HMG-CoA lyase was the first to be described, and catalyzes the cleavage of 3-hydroxy-3-methylglutaryl CoA to acetoacetate and acetyl-CoA, the common final step in ketogenesis and leucine catabolism. This protein is mainly expressed in the liver and its function is metabolic, since it produces ketone bodies as energetic fuels when glucose levels are low. Another isoform is encoded by the same gene for the mitochondrial HMG-CoA lyase (HMGCL), but it is located in peroxisomes. The last HMG-CoA lyase to be described is encoded by a different gene, HMGCLL1, and is located in the cytosolic side of the endoplasmic reticulum membrane. Some activity assays and tissue distribution of this enzyme have shown the brain and lung as key tissues for studying its function. Although the roles of the peroxisomal and cytosolic HMG-CoA lyases remain unknown, recent studies highlight the role of ketone bodies in metabolic remodeling, homeostasis, and signaling, providing new insights into the molecular and cellular function of these enzymes

Evaluating face2gene as a tool to identify cornelia de lange syndrome by facial phenotypes

Characteristic or classic phenotype of Cornelia de Lange syndrome (CdLS) is associated with a recognisable facial pattern. However, the heterogeneity in causal genes and the presence of overlapping syndromes have made it increasingly difficult to diagnose only by clinical features. DeepGestalt technology, and its app Face2Gene, is having a growing impact on the diagnosis and management of genetic diseases by analysing the features of affected individuals. Here, we performed a phenotypic study on a cohort of 49 individuals harbouring causative variants in known CdLS genes in order to evaluate Face2Gene utility and sensitivity in the clinical diagnosis of CdLS. Based on the profile images of patients, a diagnosis of CdLS was within the top five predicted syndromes for 97.9% of our cases and even listed as first prediction for 83.7%. The age of patients did not seem to affect the prediction accuracy, whereas our results indicate a correlation between the clinical score and affected genes. Furthermore, each gene presents a different pattern recognition that may be used to develop new neural networks with the goal of separating different genetic subtypes in CdLS. Overall, we conclude that computer-assisted image analysis based on deep learning could support the clinical diagnosis of CdLS.Spanish Ministry of Science, Innovation and Universities/State Research Agency RTC-2017-6494-1; RTI2018-094434-B-I00 (MCIU/AEI/FEDER, UE) to P.G.-P.; Diputación General de Aragón - FEDER: European Social Fund [Grupo de Referencia B32_17R, to J.P.] as well as funds from the European JPIAMR-VRI network “CONNECT” to P.G.-P.; Medical Faculty of the University of Lübeck J09-2017 to I. P.; German Federal Ministry of Education and Research (BMBF

Association study of lipoprotein(a) genetic markers, traditional risk factors, and coronary heart disease in HIV-1-infected patients.

Objectives: General population studies have shown associations between copy number variation (CNV) of the LPA gene Kringle-IV type-2 (KIV-2) coding region, single-nucleotide polymorphism (SNP) rs6415084 in LPA and coronary heart disease (CHD). Because risk factors for HIV-infected patients may differ from the general population, we aimed to assess whether these potential associations also occur in HIV-infected patients. Methods: A unicenter, retrospective, case-control (1:3) study. Eighteen HIV-patients with confirmed diagnosis of acute myocardial infarction (AMI) were adjusted for age, gender, and time since HIV diagnosis to 54 HIV-patients without CHD. After gDNA extraction from frozen blood, both CNV and SNP genotyping were performed using real-time quantitative PCR. All genetic and non-genetic variables for AMI were assessed in a logistic regression analysis. Results: Our results did not confirm any association in terms of lipoprotein(a) LPA structural genetic variants when comparing KIV-2 CNV (p = 0.67) and SNP genotypes (p = 0.44) between AMI cases and controls. However, traditional risk factors such as diabetes mellitus, hypertension, and CD4(+) T cell count showed association (p < 0.05) with CHD. Conclusion: Although significant associations of AMI with diabetes, hypertension and CD4(+) T cell count in HIV-patients were found, this study could not confirm the feasibility neither of KIV-2 CNV nor rs6415084 in LPA as genetic markers of CHD in HIV-infected patients.Highlights:● Individuals with HIV infection are at higher risk of coronary heart disease (CHD) than the non-infected population.● Our results showed no evidence of LPA structural genetic variants associated with CHD in HIV-1-infected patients.● Associations were found between diabetes mellitus, arterial hypertension, CD4(+) T cell count, and CHD.● The clinical usefulness of these biomarkers to predict CHD in HIV-1-infected population remains unproven.● Further studies are needed to assess the contribution of common genetic variations to CHD in HIV-infected individuals

Heart disease characterization and myocardial strain analysis in patients with PACS1 Neurodevelopmental Disorder

Background: PACS1 neurodevelopmental disorder (PACS1-NDD) (MIM# 615009) is a rare autosomal dominant disease characterized by neurodevelopmental delay, dysmorphic facial features, and congenital malformations. Heart disease (HD) is frequently present in individuals with PACS1-NDD, but a compressive review of these anomalies and an evaluation of cardiac function in a cohort of patients are lacking. Methods: (i) Cardiac evaluation in 11 PACS1-NDD patients was conducted using conventional echocardiography. (ii) Heart function was assessed by tissue Doppler imaging, and two-dimensional speckle tracking was performed in seven patients and matched controls. (iii) This systematic review focused on determining HD prevalence in individuals with PACS1-NDD. Results: In our cohort, 7 of 11 patients presented HD. (Among them, three cases of ascending aortic dilatation (AAD) were detected and one mitral valve prolapse (MVP).) None of the patients showed echocardiographic pathological values, and the left global longitudinal strain was not significantly different between patients and controls (patients −24.26 ± 5.89% vs. controls −20.19 ± 1.75%, p = 0.3176). In the literature review, almost 42% (42/100) of individuals with PACS1-NDD reportedly experienced HD. Septal defects were the most common malformation, followed by patent ductus arteriosus. Conclusions: Our results show a high prevalence of HD in PACS1-NDD patients; in this way, AAD and MVP are reported for the first time in this syndrome. Furthermore, a detailed cardiac function evaluation in our cohort did not reveal evidence of cardiac dysfunction in individuals with PACS1-NDD. Cardiology evaluation should be included for all individuals with Schuurs-Hoeijmakers syndrome

The G428A nonsense mutation in FUT2 provides strong but not absolute protection against symptomatic GII.4 Norovirus infection.

In November 2004, 116 individuals in an elderly nursing home in El Grao de Castellón, Spain were symptomatically infected with genogroup II.4 (GII.4) norovirus. The global attack rate was 54.2%. Genotyping of 34 symptomatic individuals regarding the FUT2 gene revealed that one patient was, surprisingly, a non-secretor, hence indicating secretor-independent infection. Lewis genotyping revealed that Lewis-positive and negative individuals were susceptible to symptomatic norovirus infection indicating that Lewis status did not predict susceptibility. Saliva based ELISA assays were used to determine binding of the outbreak virus to saliva samples. Saliva from a secretor-negative individual bound the authentic outbreak GII.4 Valencia/2004/Es virus, but did not in contrast to secretor-positive saliva bind VLP of other strains including the GII.4 Dijon strain. Amino acid comparison of antigenic A and B sites located on the external loops of the P2 domain revealed distinct differences between the Valencia/2004/Es and Dijon strains. All three aa in each antigenic site as well as 10/11 recently identified evolutionary hot spots, were unique in the Valencia/2004/Es strain compared to the Dijon strain. To the best of our knowledge, this is the first example of symptomatic GII.4 norovirus infection of a Le(a+b-) individual homozygous for the G428A nonsense mutation in FUT2. Taken together, our study provides new insights into the host genetic susceptibility to norovirus infections and evolution of the globally dominating GII.4 viruses.Original Publication: Beatrice Carlsson, Elin Kindberg, Javier Buesa, Gustaf E Rydell, Marta Fos Lidón, Rebeca Montava, Reem Abu Mallouh, Ammi Grahn, Jesús Rodríguez-Díaz, Juan Bellido, Alberto Arnedo, Göran Larson and Lennart Svensson, The G428A nonsense mutation in FUT2 provides strong but not absolute protection against symptomatic GII.4 Norovirus infection., 2009, PLoS ONE, (4), 5, e5593. http://dx.doi.org/10.1371/journal.pone.0005593 Licensed under Creative Common

Things are not always what they seem: From Cornelia de Lange to KBG phenotype in a girl with genetic variants in NIPBL and ANKRD11.

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Molecular Basis of the Schuurs–Hoeijmakers Syndrome: What We Know about the Gene and the PACS-1 Protein and Novel Therapeutic Approaches

The Schuurs–Hoeijmakers syndrome (SHMS) or PACS1 Neurodevelopment Disorder (PACS1-NDD) is a rare autosomal dominant disease caused by mutations in the PACS1 gene. To date, only 87 patients have been reported and, surprisingly, most of them carry the same variant (c.607C&gt;T; p.R203W). The most relevant clinical features of the syndrome include neurodevelopment delay, seizures or a recognizable facial phenotype. Moreover, some of these characteristics overlap with other syndromes, such as the PACS2 or Wdr37 syndromes. The encoded protein phosphofurin acid cluster sorting 1 (PACS-1) is able to bind to different client proteins and direct them to their subcellular final locations. Therefore, although its main function is protein trafficking, it could perform other roles related to its client proteins. In patients with PACS1-NDD, a gain-of-function or a dominant negative mechanism for the mutated protein has been suggested. This, together with the fact that most of the patients carry the same genetic variant, makes it a good candidate for novel therapeutic approaches directed to decreasing the toxic effect of the mutated protein. Some of these strategies include the use of antisense oligonucleotides (ASOs) or targeting of its client proteins

InSAR-Based Mapping to Support Decision-Making after an Earthquake

It has long been recognized that earthquakes change the stress in the upper crust around the fault rupture and can influence the behaviour of neighbouring faults and volcanoes. Rapid estimates of these stress changes can provide the authorities managing the post-disaster situation with valuable data to identify and monitor potential threads and to update the estimates of seismic and volcanic hazard in a region. Here we propose a methodology to evaluate the potential influence of an earthquake on nearby faults and volcanoes and create easy-to-understand maps for decision-making support after large earthquakes. We apply this methodology to the Mw 7.8, 2016 Ecuador earthquake. Using Sentinel-1 Interferometric Synthetic Aperture Radar (InSAR) and continuous GPS data, we measure the coseismic ground deformation and estimate the distribution of slip over the fault rupture. We also build an alternative source model using the Global Centroid Moment Tensor (CMT) solution. Then we use these models to evaluate changes of static stress on the surrounding faults and volcanoes and produce maps of potentially activated faults and volcanoes. We found, in general, good agreement between our maps and the seismic and volcanic events that occurred after the Pedernales earthquake. We discuss the potential and limitations of the methodology.This work is supported by the European Commission, Directorate-General Humanitarian Aid and Civil Protection (ECHO) under the SAFETY (Sentinel for Geohazards regional monitoring and forecasting) project (ECHO/SUB/2015/718679/Prev02) and by the Spanish Ministry of Economy and Competitiveness under INTERGEOSIMA (CGL2013-47412) and ACTIVESTEP (CGL2017-83931-C3), QUAKESTEP (1-P) + 3GEO(2-P) + GEOACTIVA (3-P) projects