146 research outputs found

    Prices and Market Structure: An Empirical Analysis of the Supermarket Industry in Chile

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    This paper investigates empirically the relationship between market structure and consumer prices in the supermarket industry in Chile. A panel of monthly data from 16 cities in the period January 1998–September 2006 was used. It was found that the more concentrated the industry is in a city, the higher the prices, while the participation of major national chains in cities tends to lower prices. Moreover, the dominant local chain was found to behave differently depending on whether or not one of the national chains was present in the city. Finally, we find that prices rise when a national chain acquires another chain and both were previously in a city (inmerge) while if only one of the two was present (outmerge), prices fall.Prices, retail, market structure

    Prevención de errores congénitos del metabolismo

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    ¿Qué es una enfermedad molecular?

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    Se conocen más de 500 enfermedades metabólicas hereditarias (EMH) definidas hasta el momento. En su conjunto estas enfermedades tienen un gran impacto sobre la salud en términos de seguridad reproductiva, morbilidad y mortalidad en el periodo perinatal, infantil y en la edad adulta

    Clima Organizacional y la Calidad de Servicio en el Hospital Geriátrico San José PNP, 2018.

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    El propósito fue contribuir a comprobar los causantes que trasciende de un adecuado servicio de salud y su clima organizacional como factor decisivo en la política publica de salud , el objetivo fue determinar el nivel mas frecuente del clima organizacional del personal administrativo y asistencia y porcentaje de usuarios satisfechos en consultorio externo, hospitalizacion y emergencia . Metodología, investigación descriptiva comparativa , no experimental , cuantitativa , muestra de 214 servidores para el clima organizacional y 300 pacientes mayores de 60 años. Resultados, en el clima organizacional , dimensión potencial humano, tiene un nivel medianamente adecuado en un 68% , en la dimensión cultura organizacional es nivel es adecuado con un 51% , en la dimensión diseño organizacional, un nivel medianamente adecuado el 54%. En la calidad de servicio, el nivel de satisfacción del cliente adulto mayor atendido en el servicio de consulta externa se encuentra 73.2% en un nivel aceptable, el nivel de satisfacción del usuario en el servicio de hospitalizacion se encuentra en 22.3% en un nivel por mejorar y el nivel de satisfacción del usuario del adulto mayor atendido en el servicio de emergencia se encuentra 45.7% en un nivel en proceso. Conclusion, el nivel clima organizacional del trabajador administrativo y asistencial , es medianamente adecuado con un 72% , el nivel de satisfacción del cliente adulto mayor, se encuentra en procesos de mejora de la calidad, observándose un 52.93% de usuarios Satisfechos.Tesi

    Endoplasmic reticulum stress and autophagy in homocystinuria patients with remethylation defects

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    Proper function of endoplasmic reticulum (ER) and mitochondria is crucial for cellular homeostasis, and dysfunction at either site as well as perturbation of mitochondria-associated ER membranes (MAMs) have been linked to neurodegenerative and metabolic diseases. Previously, we have observed an increase in ROS and apoptosis levels in patientderived fibroblasts with remethylation disorders causing homocystinuria. Here we show increased mRNA and protein levels of Herp, Grp78, IPR1, pPERK, ATF4, CHOP, asparagine synthase and GADD45 in patient-derived fibroblasts suggesting ER stress and calcium perturbations in homocystinuria. In addition, overexpressed MAM-associated proteins (Grp75, σ-1R and Mfn2) were found in these cells that could result in mitochondrial calcium overload and oxidative stress increase. Our results also show an activation of autophagy process and a substantial degradation of altered mitochondria by mitophagy in patientderived fibroblasts. Moreover, we have observed that autophagy was partially abolished by antioxidants suggesting that ROS participate in this process that may have a protective role. Our findings argue that alterations in Ca homeostasis and autophagy may contribute to the development of this metabolic disorder and suggest a therapeutic potential in homocystinuria for agents that stabilize calcium homeostasis and/or restore the proper function of ER-mitochondria communications.Ministerio de Ciencia e Innovación (SAF2010-15284 to ER), Ministerio de Economía y Competividad: Instituto de Salud Carlos III (PI13/01239 to BP), MITOLAB (S2010/BMD-2402 to BP), Ministerio de Economía y Competividad (SAF2013-43005-R to ER and LRD), and an Institutional grant from Fundación Ramón Areces to the Centro de Biología Molecular “Severo Ochoa”Peer Reviewe

    Antimicrobial, Antioxidant, and Immunomodulatory Properties of Essential Oils: A Systematic Review

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    Essential oils (EOs) are a mixture of natural, volatile, and aromatic compounds obtained from plants. In recent years, several studies have shown that some of their benefits can be attributed to their antimicrobial, antioxidant, anti-inflammatory, and also immunomodulatory properties. Therefore, EOs have been proposed as a natural alternative to antibiotics or for use in combination with antibiotics against multidrug-resistant bacteria in animal feed and food preservation. Most of the results come from in vitro and in vivo studies; however, very little is known about their use in clinical studies. A systematic and comprehensive literature search was conducted in PubMed, Embase®, and Scopus from December 2014 to April 2019 using di erent combinations of the following keywords: essential oils, volatile oils, antimicrobial, antioxidant, immunomodulation, and microbiota. Some EOs have demonstrated their efficacy against several foodborne pathogens in vitro and model food systems; namely, the inhibition of S. aureus, V. cholerae, and C. albicans has been observed. EOs have shown remarkable antioxidant activities when used at a dose range of 0.01 to 10 mg/mL in cell models, which can be attributed to their richness in phenolic compounds. Moreover, selected EOs exhibit immunomodulatory activities that have been mainly attributed to their ability to modify the secretion of cytokines.Part of the research currently in progress in the authors’ laboratory is funded by the company PAYMSA, S. A. through the grant #4501 managed by the Fundacion General Empresa-Universidad de Granada

    Generation and characterization of a human iPSC line from a patient with propionic acidemia due to defects in the PCCA gene

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    Human induced pluripotent stem cell (iPSC) line was generated from fibroblasts of a patient with propionic acidemia carrying mutations in the PCCA gene: c.1899+4_1899+7delAGTA; p.(Cys616_Val633del) and c.1430 −−?_1643+?del; p.(Gly477Glufs*9). Reprogramming factors OCT3/4, SOX2, KLF4 and c-MYC were delivered using a non-integrative method based on the Sendai virus. Once established, iPSCs have shown full pluripotency, differentiation capacity and genetic stabilityThis work was supported by Spanish Ministry of Economy and Competitiveness and European Regional Development Fund (grant numbers SAF2013-43005-R and SAF2016-76004-R). The authors thank INDEPF (Instituto de investigación y desarrollo social de enfermedades poco frecuentes), and E.Mansilla for her excellent assistance in the karyotype analysis (Instituto de Genética Médica y Molecular del Hospital Universitario de La Paz, Madrid, Spain). Centro de Biología Molecular Severo Ochoa receives an institutional grant from Fundación Ramón Areces (grant number CNXVII

    Mapas de amenaza volcánica mediante simulaciones de caída de Tefra del volcán Telíca a partir del análisis de perfiles de viento del año 2019

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    La presente investigación, tuvo como objetivo principal proponer mapas de amenaza volcánica mediante simulaciones por caída de Tefra del volcán Telíca a partir del análisis de perfiles de viento del año 2019. Ubicado en el departamento de León, municipio de Telíca. Para concretar este objetivo principal se plantearon los siguientes objetivos específicos que implicaron analizar los perfiles de vientos del volcán Telíca a lo largo del año 2019 con un comportamiento meteorológicamente estable, para posteriormente, simular la dispersión de la Tefra volcánica durante cada mes del año 2019 con el software Tephra 2 con un índice de explosividad volcánica de 2 y 4, y finalmente, desarrollar información que contribuya a fortalecer los planes de gestión de riesgos volcánicos, de manera que permita elaborarlos a mayor detalle. La investigación se desarrolló a partir de la compilación de información y datos del volcán Telíca. Primeramente a través de la recolección de datos numéricos mediante sondeos de vientos semanales del volcán Telíca, que permitieron conocer las diferentes velocidades según la altura, posteriormente se generaron rosetas de viento, histogramas 3D y los perfiles de viento en función de las variables múltiples como la velocidad, altura y dirección del viento, de forma que, se logró observar los diferentes patrones de dispersión para su análisis y descripción. Por consiguiente, se procesaron los perfiles de viento con el software Tephra 2 obteniendo los mapas de Isopacas. Por último, se digitalizaron con la ayuda del software ArcGis 10.5, logrando como resultados las simulaciones de dispersión de tefra. En conclusión, de acuerdo a su índice de explosividad, la dispersión se da mayormente hacia el Oeste del volcán Telíca, afectando diferentes poblados, en su mayoría, los más cercanos al volcán. Palabras Claves: Geología, Vulcanología, Caída de Tefra, Volcán Telíca, Simulación computacional, Tephra 2

    Identification of 34 novel mutations in propionic acidemia: Functional characterization of missense variants and phenotype associations

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    Propionic acidemia (PA) is caused by mutations in the PCCA and PCCB genes, encoding α and β subunits, respectively, of the mitochondrial enzyme propionyl-CoA carboxylase (PCC). Up to date, >200 pathogenic mutations have been identified, mostly missense defects. Genetic analysis in PA patients referred to the laboratory for the past 15 years identified 20 novel variants in the PCCA gene and 14 in the PCCB gene. 21 missense variants were predicted as probably disease-causing by different bioinformatics algorithms. Structural analysis in the available 3D model of the PCC enzyme indicated potential instability for most of them. Functional analysis in a eukaryotic system confirmed the pathogenic effect for the missense variants and for one amino acid deletion, as they all exhibited reduced or null PCC activity and protein levels compared to wild-type constructs. PCCB variants p.E168del, p.Q58P and p.I460T resulted in medium-high protein levels and no activity. Variants p.R230C and p.C712S in PCCA, and p.G188A, p.R272W and p.H534R in PCCB retained both partial PCC activity and medium-high protein levels. Available patients-derived fibroblasts carriers of some of these mutations were grown at 28 °C or 37 °C and a slight increase in PCC activity or protein could be detected in some cases at the folding-permissive conditions. Examination of available clinical data showed correlation of the results of the functional analysis with disease severity for most mutations, with some notable exceptions, confirming the notion that the final phenotypic outcome in PA is not easily predictedWe thank the following physicians/clinicians for sending samples for genetic analysis: Dr. Wilson (Auckland, New Zealand), Dr. Parini (Rome, Italy), Dr. Vilaseca (Barcelona, Spain), Dr. Gockay (Istanbul, Turkey), Dr. Al Sannaa (Saudi Arabia), Dr. Pedrón (Madrid, Spain), Dr. Savvapoulou (Tesalonica, Greece), Dr. Martínez-Pardo (Madrid, Spain), Dr. Lama (Madrid, Spain), Dr. Lemes (Montevideo, Uruguay), Dr. Van Calcar (Madison, USA), Dr. Pintos (Badalona, Spain), Dr. Laszlo (Szeged, Hungary), Dr. Kuijtmans (Nijmegen, The Netherlands), Dr. Schatz (München, Germany), Dr. EL Khateeb (Jordan), Dr. de las Heras (Baracaldo, Spain), Dr. Miñana (Buenos Aires, Argentina). The technical assistance of A. Sánchez is gratefully acknowledged. This work was supported by Spanish Ministry of Economy and Competitiveness and European Regional Development Fund (grant number SAF2016-76004-R). Centro de Biología Molecular Severo Ochoa receives an institutional grant from Fundación Ramón Arece

    Pathogenic implications of dysregulated miRNAs in propionic acidemia related cardiomyopathy

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    Cardiac alterations (hypertrophic/dilated cardiomyopathy, and rhythm alterations) are one of the major causes of mortality and morbidity in propionic acidemia (PA), caused by the deficiency of the mitochondrial enzyme propionyl-CoA carboxylase (PCC), involved in the catabolism of branched-chain amino acids, cholesterol, and odd-chain fatty acids. Impaired mitochondrial oxidative phosphorylation has been documented in heart biopsies of PA patients, as well as in the hypomorphic Pcca−/−(A138T) mouse model, in the latter correlating with increased oxidative damage and elevated expression of cardiac dysfunction biomarkers atrial and brain natriuretic peptides (ANP and BNP) and beta-myosin heavy chain (β-MHC). Here we characterize the cardiac phenotype in the PA mouse model by histological and echocardiography studies and identify a series of upregulated cardiac-enriched microRNAs (miRNAs) in the PA mouse heart, some of them also altered as circulating miRNAs in PA patients’ plasma samples. In PA mice hearts, we show alterations in signaling pathways regulated by the identified miRNAs, which could be contributing to cardiac remodeling and dysfunction; notably, an activation of the mammalian target of rapamycin (mTOR) pathway and a decrease in autophagy, which are reverted by rapamycin treatment. In vitro studies in HL-1 cardiomyocytes indicate that propionate, the major toxic metabolite accumulating in the disease, triggers the increase in expression levels of miRNAs, BNP, and β-MHC, concomitant with an increase in reactive oxygen species. Our results highlight miRNAs and signaling alterations in the PCC-deficient heart which may contribute to the development of PA-associated cardiomyopathy and provide a basis to identify new targets for therapeutic interventionThis work was supported by Spanish Ministry of Economy and Competitiveness and European Regional Development Fund (grant number SAF2016-76004-R) and by Fundación Isabel Gemio and Fundación La Caixa (LCF/PR/PR16/ 11110018). AFG is funded by the FPI-UAM program, EAB and ARB by the Spanish Ministry of Science, Innovation and Universities (predoctoral fellowships FPU15/02923 and BES-2014-069420, respectively
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