145 research outputs found

    Nieuwe wijn in oude zakken, of toch niet? : de Bolognaverklaring in historisch perspectief

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    Проблеми та перспективи етнологічного дослідження меморіальних пам’ятників Білорусі XIX – початку ХХІ століття

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    The article reviews main directions and results of investigation of Byelorussian memorial monuments from XIXth to the beginning of the XXIth century. It observes problems and peculiarities of the methodology of research in the conditions of sick level of it’s elaboration. There were signed out three main segments of the research: rural cemeteries of the XXth – beginning of the XXIth century, town necropolis of the same period and cemeteries of the second third of the XXth – beginning of the XXIth century. Main transformations of the memorial monuments are explained throug the functionalist conceptions. Special attention is devoted to the semantical segment of the research as to the most favourable for realising that changes what took part in the XXth century. We also mark the problems of a family and individuality as to one of the midpoints of the memorial monumen’s forming.Артыкул прысвечаны агляду асноўных накірункаў і вынікаў даследавання мемарыяльных помнікаў Беларусі 19 – пач. 21 ст. Разглядяюцца праблемы і асаблівасці метадалогіі даследавання ва умовах слабай распрацаванасці цемы. Вылучаны тры асноўныя семгенты даследавання: вясковыя могілкі 19 – пач. 20 ст, гарадскія некропалі таго ж перыяду, і некропалі 2 трэцці 20 – 21 стагоддзяў. Асноўныя трансфармацыі мемарыяльных помнікаў трактуюца зходзячы з функцыяналістскіх канцэпцый, асаблівая увага удзяляецца семантычнай саставляючай даследавання, як наібольш, на думку аўтара, прадуктыўнай для разумення ўнутраных прычын тых трансфармацый, якія адбыліся у 20 стагоддзі. Асаблівая увага удзелена праблеме сям'і і індывідуальнасці як адным з ключавых фактараў у формавані мемарыяльных помнікаў

    Muscular dystrophy with arrhythmia caused by loss-of-function mutations in BVES

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    Objective To study the genetic and phenotypic spectrum of patients harboring recessive mutations in BVES. Methods We performed whole-exome sequencing in a multicenter cohort of 1929 patients with a suspected hereditary myopathy, showing unexplained limb-girdle muscular weakness and/or elevated creatine kinase levels. Immunohistochemistry and mRNA experiments on patients' skeletal muscle tissue were performed to study the pathogenicity of identified loss-of-function (LOF) variants in BVES. Results We identified 4 individuals from 3 families harboring homozygous LOF variants in BVES, the gene that encodes for Popeye domain containing protein 1 (POPDC1). Patients showed skeletal muscle involvement and cardiac conduction abnormalities of varying nature and severity, but all exhibited at least subclinical signs of both skeletal muscle and cardiac disease. All identified mutations lead to a partial or complete loss of function of BVES through nonsense-mediated decay or through functional changes to the POPDC1 protein. Conclusions We report the identification of homozygous LOF mutations in BVES, causal in a young adult-onset myopathy with concomitant cardiac conduction disorders in the absence of structural heart disease. These findings underline the role of POPDC1, and by extension, other members of this protein family, in striated muscle physiology and disease. This disorder appears to have a low prevalence, although it is probably underdiagnosed because of its striking phenotypic variability and often subtle yet clinically relevant manifestations, particularly concerning the cardiac conduction abnormalities

    Rise of the Digitized Public Intellectual: Death of the Professor in the Network Neutral Internet Age

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    The centralised discourse claiming ownership of ‘knowledge’ and ‘higher education’ seems to be declining as the decentralising discourse extolling open source software and informal social network communication are emerging: yet the two are complementary when higher education is seen as a commodity. Thus, in the internet age of the twenty first century there is no consistent narrative to identify what a ‘higher education’ consists of. J. F. Lyotard famously predicted in The Post Modern Condition that the commercialised computer age would ‘sound the knell’ of the professor. Lyotard understood that in order to begin to philosophise about higher education in an era of computerisation, the gatekeeper of knowledge role traditionally attributed to professors through a university title must first be rendered illegitimate. Lyotard did not envision, however, what a higher education might look like within the network neutral internet space, where the difference between ‘higher’ and ‘public’ education can be reduced through open accessibility to, and shared construction of, knowledge. Embracing a Socratic model of public discourse that openly challenges an epistemology of consensus, network neutrality has the potential to redefine the role of professors as fiduciaries of education across society, even globally. The resulting academic equality between professors and the public recreates the university as a boundless meeting space for public dialogue, and the professor as a digitized public intellectual

    Introduction: Understanding the Dutch Golden Age

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    The Armed Forces

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    A Politico-Communal Reading of the Rose

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    Lettura del Fiore in rapporto alle fonti retoriche e politiche di ambiente comunal
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