1000 resultados para Routh, Martin Joseph, 1755-1854.
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Donateur : Bonaparte, Roland (1858-1924)
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[Vente (Livres). 1854-12-18. Paris]
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[Vente (Livres). 1854-06-19. Paris]
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Machado-Joseph disease is the most frequently found dominantly-inherited cerebellar ataxia. Over-repetition of a CAG trinucleotide in the MJD1 gene translates into a polyglutamine tract within the ataxin 3 protein, which upon proteolysis may trigger Machado-Joseph disease. We investigated the role of calpains in the generation of toxic ataxin 3 fragments and pathogenesis of Machado-Joseph disease. For this purpose, we inhibited calpain activity in mouse models of Machado-Joseph disease by overexpressing the endogenous calpain-inhibitor calpastatin. Calpain blockage reduced the size and number of mutant ataxin 3 inclusions, neuronal dysfunction and neurodegeneration. By reducing fragmentation of ataxin 3, calpastatin overexpression modified the subcellular localization of mutant ataxin 3 restraining the protein in the cytoplasm, reducing aggregation and nuclear toxicity and overcoming calpastatin depletion observed upon mutant ataxin 3 expression. Our findings are the first in vivo proof that mutant ataxin 3 proteolysis by calpains mediates its translocation to the nucleus, aggregation and toxicity and that inhibition of calpains may provide an effective therapy for Machado-Joseph disease.
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This edited volume examines, from a ritual perspective, Pentecostal-Charismatic groups that are the fastest growing religious movements in the world today. The authors, who are anthropologists, ethnologists or sociologists (with one theologian) collected rich and diverse material on healing, deliverance, personal devotion, public engagement. Their work covers several regions such as Chile, South California, Fiji, Kenya, and Sweden. After an introduction by the editor, eleven chapters examine various issues relevant to the field. Overcoming the diversity of subjects, the unity of the volume is provided by the general ritual perspective and by the methodological implications of employing such a perspective.
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Joseph Maria Valls i Vicens was an important Catalan intellectual who had an important performance in literature, politics , sociology, pedagogy and several cultural initiatives. The author focalizes the study of the young writer's thought as a common character of a wide sector of intellectuals that had developed an important activity in local politics and culture all through the period.