1000 resultados para Steere, Joseph Beal, 1842-1940


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[spa] El artículo trata de la retórica del periodismo y la comunicación institucional en la localidad de Manlleu (Barcelona, Spain). El boletín municipal Manlleu (1940-1957) fue una cabecera local en el franquismo de posguerra que publicó los programas de fiesta mayor. El estudio muestra cómo creó la propaganda política una memoria histórica con el martirologio y los agravios de la República. Esta investigación forma parte de los estudios sobre fiesta y discurso Celebratio et oratio. Y contribuye a los repertorios históricos de comunicación local e institucional (ReCoLI). El repertorio combina los ámbitos del discurso, las instituciones locales y la ideología, bajo una perspectiva histórica. "Institutional communication and local press during the Franco regime in Manlleu (Spain, 1940-1957)".

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Machado-Joseph disease or Spinocerebellar ataxia type 3 is a progressive fatal neurodegenerative disorder caused by the polyglutamine-expanded protein ataxin-3. Recent studies demonstrate that RNA interference is a promising approach for the treatment of Machado-Joseph disease. However, whether gene silencing at an early time-point is able to prevent the appearance of motor behavior deficits typical of the disease when initiated before onset of the disease had not been explored. Here, using a lentiviral-mediated allele-specific silencing of mutant ataxin-3 in an early pre-symptomatic cerebellar mouse model of Machado-Joseph disease we show that this strategy hampers the development of the motor and neuropathological phenotypic characteristics of the disease. At the histological level, the RNA-specific silencing of mutant ataxin-3 decreased formation of mutant ataxin-3 aggregates, preserved Purkinje cell morphology and expression of neuronal markers while reducing cell death. Importantly, gene silencing prevented the development of impairments in balance, motor coordination, gait and hyperactivity observed in control mice. These data support the therapeutic potential of RNA interference for Machado-Joseph disease and constitute a proof of principle of the beneficial effects of early allele-specific silencing for therapy of this disease.