Abro la ventana y veo... 'cómo el niño de Educación Infantil descubre el mundo que le rodea con la fuerza del cariño'.

Monográfico con el título: 'Educación Infantil'

La disciplina del buen uso muscular en la actividad instrumental.

Las circunstancias propias del instrumentista de conservatorio son propicias para que se den desórdenes de uso muscular. En el presente artículo se analizan los principales problemas que sufren estos profesionales, se relacionan personalidades y entidades que realizan actividades para combatir las tecnopatías del músico y se aboga por la creación de disciplinas en los conservatorios para la prevención de factores de riesgo para el músico.

El IES Miguel Catalán : la fuerza de la normalidad

Resumen basado en el de la publicación.

Les pedagogies de la masculinitat : Thomas Arnold i el ???Muscular Christianity???

Resumen basado en el de la publicaci??n

La fuerza normativa de la Constitución ecuatoriana actual (Artículos Varios)

Este trabajo está encaminado a analizar concisamente las condiciones de exigibilidad y aplicabilidad de las normas constitucionales vigentes en el Ecuador, a la luz de varias concepciones teóricas y doctrinarias actuales que analizan su función normativa. la Constitución es estudiada como norma jurídica y no como simple enunciado de buenos deseos o programa político, así, el autor aborda las consecuencias de la aplicación directa e inmediata de sus preceptos, indaga los valores, principios e instituciones que positiviza y su estimación como fuente de derecho, trata de la interpretación de conformidad con la Constitución y sus funciones: la legitimación y la limitación del poder, la defensa de la libertad, etc. Refiere además las relaciones entre Constitución, derecho internacional e instrumentos de integración. Para lograr estos objetivos acude a jurisprudencia ecuatoriana relacionada.

Alcance y contenido de los INCOTERMS ICC 2010 utilizados en la adquisición de bienes de carácter estratégico por la fuerza terrestre

El mal uso de los INCOTERMS puede tener implicaciones jurídicas y comerciales, aumento de costos, seguros, formas de pago, fiscalidad, de contratación, etc. A través de la presente tesis, estructurada en tres capítulos, nos referimos a esta problemática. El Capítulo I de determina la normativa legal vigente para adquirir bienes en general y de tipo estratégico; así como también da a conocer los aspectos más relevantes del planeamiento logístico que la Fuerza Terrestre efectúa para la obtención de estos bienes, a fin de mantener y acrecentar su capacidad combativa en toda circunstancia y escenario en el que actúa. El Capítulo II de esta tesis hace un análisis del origen del organismo internacional que aprobó el Contrato de Compra Venta Internacional de Mercancías; que se constituye en un conjunto de normas que se refieren a tipos particulares de mercancías, que necesitan tratamiento o autorizaciones especiales. Además, determina cuáles son las reglas y usos uniformes del Comercio Internacional, la categorización de los INCOTERMS, sus definiciones y alcances. Finalmente, en el Capítulo III se evidencian las principales cláusulas que deberán incluirse según los INCOTERMS utilizados por la Fuerza Terrestre y de sus particularidades.

La inteligencia emocional, según Daniel Goleman y, el liderazgo transformador, según Bernard Bass, como elementos clave en la formación y desarrollo de equipos efectivos en la fuerza comercial de microfinanzas CREDI FE Zona Quito

El desarrollo de la presente tesis busca la comprensión del aporte que brindan los conceptos: inteligencia emocional y liderazgo transformador en la formación y desarrollo de equipos efectivos de trabajo. Ambos elementos son relevantes para lograr una buena gestión de trabajo individual y de equipo, que favorezca la eficiencia organizacional y las relaciones interpersonales. Desde la perspectiva laboral, tanto el liderazgo transformador como la inteligencia emocional generan en las personas sentido de pertenencia a la organización, promueven su desarrollo profesional y personal, mejoran su satisfacción y actitud logrando un adecuado trabajo de equipo. La inteligencia emocional es la capacidad de entender los sentimientos propios y de los demás para generar respuestas adecuadas. El liderazgo transformador es el conjunto de habilidades y destrezas de un individuo para gestionar, estimular o motivar a un equipo favoreciendo el cumplimiento de objetivos comunes. Finalmente, a través de la correlación entre las variables inteligencia emocional y liderazgo transformador, se buscará entender el nivel de efectividad de los equipos de alto rendimiento en la fuerza comercial de microfinanzas CREDI FE Zona Quito.

República Bolivariana de Venezuela: la democracia como una idea-fuerza (Tema Central)

Tras años de democracia representativa, establecida bajo los parámetros de la democracia liberal, se inicia en 1998 en Venezuela un período de oportunidades políticas que da salida a los años de lucha y desencanto social producto de la deslegitimación del sistema político, a la vez que permite la resignificación de la democracia como una idea-fuerza, persiguiendo –a través de la superación de los límites formales– dar lugar a una concepción más exigente, que se origine y se nutra de las realidades organizativas y de lucha del pueblo venezolano. Sin embargo, la resignificación de la democracia venezolana es atravesada por la idea del socialismo del siglo XXI, apuntando hacia la construcción de un camino compartido, donde las tensiones entre diversos sectores de la población, entre ésta y el gobierno, y al interior del movimiento bolivariano implican mayores retos para la consolidación de esta idea. La democracia en Venezuela supera los límites teóricos entendiendo que ésta se construye y reconstruye en lo cotidiano, y solo así podrá conformarse como un proyecto histórico de transformación social.

La administración de los bienes minerales como fuerza motriz del desarrollo social (Tema Central)

The mineral sector has an extreme strategic relevance for the social and economic development of any country. Therefore, proper management of existing mineral resources in a given area is closely linked to the full exercise of sovereignty. Thus, in pretending to guarantee efficient control of resources, the majority of the countries classify mineral resources as state property; however, because of the high cost and the risk involved, the economic exploitation of these resources is more efficient when driven by private initiative. Hence, the basic resource exploitation model is a modern legal concept in which the control over resources belongs to the State, but the direct economic exploitation of this heritage belongs to some individuals according to law and by offering the necessary social and economic counterparts.

Cell-penetrating peptide-morpholino conjugates alter pre-mRNA splicing of DMD (Duchenne muscular dystrophy) and inhibit murine coronavirus replication in vivo

The cellular uptake of PMOs (phosphorodiamidate morpholino oligomers) can be enhanced by their conjugation to arginine-rich CPPs (cell-penetrating peptides). Here, we discuss our recent findings regarding (R-Ahx-R)(4)AhxB (Ahx is 6-aminohexanoic acid and B is beta-alanine) CPP-PMO conjugates in DMD (Duchenne muscular dystrophy) and murine coronavirus research. An (R-Ahx-R)(4)AhxB-PMO conjugate was the most effective compound in inducing the correction of mutant dystrophin transcripts in myoblasts derived from a canine model of DMD. Similarly, normal levels of dystrophin expression were restored in the diaphragms of mdx mice, with treatment starting at the neonatal stage, and protein was still detecTable 22 weeks after the last dose of an (R-Ahx-R)(4)AhxB-PMO conjugate. Effects of length, linkage and carbohydrate modification of this CPP on the delivery of a PMO were investigated in a coronavirus mouse model. An (R-Ahx-R)(4)AhxB-PMO conjugate effectively inhibited viral replication, in comparison with other peptides conjugated to the same PMO. Shortening the CPP length, modifying it with a mannosylated serine moiety or replacing it with the R(9)F(2) CPP significantly decreased the efficacy of the resulting PPMO (CPP-PMO conjugate). We attribute the success of this CPP to its stability in serum and its capacity to transport PMO to RNA targets in a manner superior to that of poly-arginine CPPs.

Food restriction reverses the hyper-muscular phenotype and force generation capacity deficit of the myostatin null mouse

Food restriction has a great impact on skeletal muscle mass by inducing muscle protein breakdown to provide substrates for energy production through gluconeogenesis. Genetic models of hyper-muscularity interfere with the normal balance between protein synthesis and breakdown which eventually results in extreme muscle growth. Mutations or deletions in the myostatin gene result in extreme muscle mass. Here we evaluated the impact of food restriction for a period of 5 weeks on skeletal muscle size (i.e., fibre cross-sectional area), fibre type composition and contractile properties (i.e., tetanic and specific force) in myostatin null mice. We found that this hyper-muscular model was more susceptible to catabolic processes than wild type mice. The mechanism of skeletal muscle mass loss was examined and our data shows that the myostatin null mice placed on a low calorie diet maintained the activity of molecules involved in protein synthesis and did not up-regulate the expression of genes pivotal in ubiquitin-mediated protein degradation. However, we did find an increase in the expression of genes associated with autophagy. Surprisingly, the reduction on muscle size was followed by improved tetanic and specific force in the null mice compared to wild type mice. These data provide evidence that food restriction may revert the hyper-muscular phenotype of the myostatin null mouse restoring muscle function.

Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by ptosis, dysphagia and proximal limb weakness. Autosomal-dominant OPMD is caused by a short (GCG)8–13 expansions within the first exon of the poly(A)-binding protein nuclear 1 gene (PABPN1), leading to an expanded polyalanine tract in the mutated protein. Expanded PABPN1 forms insoluble aggregates in the nuclei of skeletal muscle fibres. In order to gain insight into the different physiological processes affected in OPMD muscles, we have used a transgenic mouse model of OPMD (A17.1) and performed transcriptomic studies combined with a detailed phenotypic characterization of this model at three time points. The transcriptomic analysis revealed a massive gene deregulation in the A17.1 mice, among which we identified a significant deregulation of pathways associated with muscle atrophy. Using a mathematical model for progression, we have identified that one-third of the progressive genes were also associated with muscle atrophy. Functional and histological analysis of the skeletal muscle of this mouse model confirmed a severe and progressive muscular atrophy associated with a reduction in muscle strength. Moreover, muscle atrophy in the A17.1 mice was restricted to fast glycolytic fibres, containing a large number of intranuclear inclusions (INIs). The soleus muscle and, in particular, oxidative fibres were spared, even though they contained INIs albeit to a lesser degree. These results demonstrate a fibre-type specificity of muscle atrophy in this OPMD model. This study improves our understanding of the biological pathways modified in OPMD to identify potential biomarkers and new therapeutic targets.

Characterisation of dystrophin in carriers of Duchenne muscular dystrophy.

Dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene, was studied in needle biopsy samples taken from the quadriceps muscle of 15 asymptomatic carriers of DMD (13 adults and 2 young girls) and one symptomatic adult carrier. Antibodies to N- and C-terminal regions of dystrophin were used for both Western blot analysis and immunocytochemistry and a monoclonal antibody to beta-spectrin used to assess membrane integrity. All asymptomatic adult carriers showed some abnormality in dystrophin immunostaining but very few negative fibres were present. A clear mosaic of dystrophin positive and negative fibres was seen only in the adult symptomatic carrier and the two young girls. On a Western blot, all carriers studied had dystrophin of normal molecular weight, but most had reduced abundance. In adult carriers, the amount of dystrophin relative to normal controls varied, but it was unrelated to age, serum creatine kinase (CK) levels or to the degree of pathology. Carriers with normal CK showed abnormalities in dystrophin expression. The dystrophin immunoblotting profile of the 2 young girls was very similar to that of their mothers, but the mosaic pattern of immunostaining was not apparent in the older carriers. In conclusion, dystrophin immunostaining and Western blot analysis of biopsy samples from asymptomatic carriers is often abnormal and they may be useful additional aids for establishing carrier status, particularly in younger girls.

Characterisation of dystrophin in fetuses at risk for Duchenne muscular dystrophy.

Dystrophin, the product of the Duchenne muscular dystrophy (DMD) gene, was studied in muscle from 16 human fetuses at risk for the disease. Eleven high risk (greater than 95% probability) and 5 low-risk (less than 25% probability) fetuses were studied with antibodies raised to different regions of the protein. All low-risk fetuses showed a similar pattern to that of normal fetuses of a comparable age: using Western blot analysis, a protein was detected of similar size and abundance to that of normal fetuses (i.e. smaller molecular weight than that of adult muscle); immunocytochemistry showed uniform sarcolemmal staining in fetuses older than 18 weeks gestation and differential staining of myotubes at different stages of development (distinguished by size) in younger fetuses (less than 15 weeks gestation). In contrast, Western blot analysis of high-risk fetuses detected low levels of dystrophin in 4 cases; 7 fetuses had no detectable protein. Immunocytochemistry with some dystrophin antibodies showed weak staining of the sarcolemma and around central nuclei in younger fetuses; in older fetuses there was little sarcolemmal staining with any antibody other than occasional positive fibres. These results indicate that careful study of dystrophin in fetuses at risk for DMD can be used to establish the clinical phenotype and provide additional information for future family counselling.

Dystrophin analysis using a panel of anti-dystrophin antibodies in Duchenne and Becker muscular dystrophy.

Dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene, was studied in 19 patients with Xp21 disorders and in 25 individuals with non-Xp21 muscular dystrophy. Antibodies raised to seven different regions spanning most of the protein were used for immunocytochemistry. In all patients specific dystrophin staining anomalies were detected and correlated with clinical severity and also gene deletion. In patients with Becker muscular dystrophy (BMD) the anomalies detected ranged from inter- and intra-fibre variation in labelling intensity with the same antibody or several antibodies to general reduction in staining and discontinuous staining. In vitro evidence of abnormal dystrophin breakdown was observed reanalysing the muscle of patients, with BMD and not that of non-Xp21 dystrophies, after it has been stored for several months. A number of patients with DMD showed some staining but this did not represent a diagnostic problem. Based on the data presented, it was concluded that immunocytochemistry is a powerful technique in the prognostic diagnosis of Xp21 muscular dystrophies.