127 research outputs found

    Utopianism for a dying planet

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    Radical changes in individual and collective behaviour may be required to mitigate the impact of climate change over the coming decades. Drawing on a new book, Gregory Claeys argues that a utopian outlook can provide the impetus for transitioning to a more sustainable way of life

    The (Not So) Unconventional Monetary Policy of the European Central Bank Since 2008 Draft

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    Directorate General for Internal Policies -- Policy Department A: Economic and Scientific Polic

    My Path to and with Orwell

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    Orwell felt that socialism (and anarchism) meant an identity of belonging based on equality. It touched him deeply, and the memory remained with him the rest of his life.Orwell felt that socialism (and anarchism) meant an identity of belonging based on equality. It touched him deeply, and the memory remained with him the rest of his life

    European Central Bank Quantitative Easing: The Detailed Manual

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    Altered splicing of the BIN1 muscle-specific exon in humans and dogs with highly progressive centronuclear myopathy

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    Amphiphysin 2, encoded by BIN1, is a key factor for membrane sensing and remodelling in different cell types. Homozygous BIN1 mutations in ubiquitously expressed exons are associated with autosomal recessive centronuclear myopathy (CNM), a mildly progressive muscle disorder typically showing abnormal nuclear centralization on biopsies. In addition, misregulation of BIN1 splicing partially accounts for the muscle defects in myotonic dystrophy (DM). However, the muscle-specific function of amphiphysin 2 and its pathogenicity in both muscle disorders are not well understood. In this study we identified and characterized the first mutation affecting the splicing of the muscle-specific BIN1 exon 11 in a consanguineous family with rapidly progressive and ultimately fatal centronuclear myopathy. In parallel, we discovered a mutation in the same BIN1 exon 11 acceptor splice site as the genetic cause of the canine Inherited Myopathy of Great Danes (IMGD). Analysis of RNA from patient muscle demonstrated complete skipping of exon 11 and BIN1 constructs without exon 11 were unable to promote membrane tubulation in differentiated myotubes. Comparative immunofluorescence and ultrastructural analyses of patient and canine biopsies revealed common structural defects, emphasizing the importance of amphiphysin 2 in membrane remodelling and maintenance of the skeletal muscle triad. Our data demonstrate that the alteration of the muscle-specific function of amphiphysin 2 is a common pathomechanism for centronuclear myopathy, myotonic dystrophy, and IMGD. The IMGD dog is the first faithful model for human BIN1-related CNM and represents a mammalian model available for preclinical trials of potential therapies

    Diagnosis and risk stratification of chest pain patients in the emergency department: focus on acute coronary syndromes. A position paper of the Acute Cardiovascular Care Association.

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    This paper provides an update on the European Society of Cardiology task force report on the management of chest pain. Its main purpose is to provide an update on the decision algorithms and diagnostic pathways to be used in the emergency department for the assessment and triage of patients with chest pain symptoms suggestive of acute coronary syndromes

    John Stuart Mill and Fourierism: ‘association’, ‘friendly rivalry’ and distributive justice

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    John Stuart Mill’s self-description as ‘under the general designation of Socialist’ has been under-explored. It is an important feature of something else often overlooked: the importance of the French context of Mill’s thought. This article focuses on the role of Fourierism in the development of Mill’s ideas, exploring the links to Fourierism in Mill’s writing on profit-sharing; his use of the words ‘association’ and ‘friendly rivalry’; and his views concerning distributive justice. It then reconsiders his assessment of Fourierism as a desirable, workable and immediately implementable form of social reform, ultimately arguing it was Mill’s most-preferred form of ‘utopian’ socialism

    After 'progress': the history of political thought and the making of a green utopia

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