27 research outputs found

    Educational reform and modernisation in Europe: The role of national contexts in mediating the new public management

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    This is the author accepted manuscript. The final version is available from SAGE Publications via the DOI in this recordThis article examines the spread of new public management (NPM) across European education systems as it has traversed national boundaries. While recognising the transnational dimensions of the spread of NPM, the authors offer new insights into the importance of national contexts in mediating this development in educational settings by focusing upon NPM within three European countries (England, Italy and Norway). We reveal its recontextualisation in these sites and the interplay between NPM, and local and national conditions. This analysis is underpinned by a theoretical framework that seeks to capture the relationship between education and the state and to reveal tensions produced by NPM both as a shaping force and an entity shaped by local conditions in these contexts. The article concludes by focusing upon the complexities and specificities of NPM recontextualisation in the three countries as a basis for a reflection upon possible future policy trajectories

    Impaired NDRG1 functions in Schwann cells cause demyelinating neuropathy in a dog model of Charcot-Marie-Tooth type 4D

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    Mutations in the N-myc downstream-regulated gene 1 (NDRG1) cause degenerative polyneuropathy in ways that are poorly understood. We have investigated Alaskan Malamute dogs with neuropathy caused by a missense mutation in NDRG1. In affected animals, nerve levels of NDRG1 protein were reduced by more than 70% (p < 0.03). Nerve fibers were thinly myelinated, loss of large myelinated fibers was pronounced and teased fiber preparations showed both demyelination and remyelination. Inclusions of filamentous material containing actin were present in adaxonal Schwann cell cytoplasm and Schmidt-Lanterman clefts. This condition strongly resembles the human Charcot-MarieTooth type 4D. However, the focally folded myelin with adaxonal infoldings segregating the axon found in this study are ultrastructural changes not described in the human disease. Furthermore, lipidomic analysis revealed a profound loss of peripheral nerve lipids. Our data suggest that the low levels of mutant NDRG1 is insufficient to support Schwann cells in maintaining myelin homeostasis. (C) 2020 The Author(s). Published by Elsevier B.V. This is an open access article under the CC BY license

    Identification of novel risk loci and causal insights for sporadic Creutzfeldt-Jakob disease: a genome-wide association study

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    Background: Human prion diseases are rare and usually rapidly fatal neurodegenerative disorders, the most common being sporadic Creutzfeldt-Jakob disease (sCJD). Variants in the PRNP gene that encodes prion protein are strong risk factors for sCJD but, although the condition has similar heritability to other neurodegenerative disorders, no other genetic risk loci have been confirmed. We aimed to discover new genetic risk factors for sCJD, and their causal mechanisms. Methods: We did a genome-wide association study of sCJD in European ancestry populations (patients diagnosed with probable or definite sCJD identified at national CJD referral centres) with a two-stage study design using genotyping arrays and exome sequencing. Conditional, transcriptional, and histological analyses of implicated genes and proteins in brain tissues, and tests of the effects of risk variants on clinical phenotypes, were done using deep longitudinal clinical cohort data. Control data from healthy individuals were obtained from publicly available datasets matched for country. Findings: Samples from 5208 cases were obtained between 1990 and 2014. We found 41 genome-wide significant single nucleotide polymorphisms (SNPs) and independently replicated findings at three loci associated with sCJD risk; within PRNP (rs1799990; additive model odds ratio [OR] 1·23 [95% CI 1·17-1·30], p=2·68 × 10-15; heterozygous model p=1·01 × 10-135), STX6 (rs3747957; OR 1·16 [1·10-1·22], p=9·74 × 10-9), and GAL3ST1 (rs2267161; OR 1·18 [1·12-1·25], p=8·60 × 10-10). Follow-up analyses showed that associations at PRNP and GAL3ST1 are likely to be caused by common variants that alter the protein sequence, whereas risk variants in STX6 are associated with increased expression of the major transcripts in disease-relevant brain regions. Interpretation: We present, to our knowledge, the first evidence of statistically robust genetic associations in sporadic human prion disease that implicate intracellular trafficking and sphingolipid metabolism as molecular causal mechanisms. Risk SNPs in STX6 are shared with progressive supranuclear palsy, a neurodegenerative disease associated with misfolding of protein tau, indicating that sCJD might share the same causal mechanisms as prion-like disorders. Funding: Medical Research Council and the UK National Institute of Health Research in part through the Biomedical Research Centre at University College London Hospitals National Health Service Foundation Trust

    Comparing effects and side effects of different school inspection systems across Europe

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    In this article, different inspection models are compared in terms of their impact on school improvement and the mechanisms each of these models generates to have such an impact. Our theoretical framework was drawn from the programme theories of six countries’ school inspection systems (i.e. the Netherlands, England, Sweden, Ireland, the province of Styria in Austria and the Czech Republic). We describe how inspection models differ in the scheduling and frequency of visits (using a differentiated or cyclical approach), the evaluation of process and/or output standards, and the consequences of visits, and how these models lead to school improvement through the setting of expectations, the use of performance feedback and actions of the school’s stakeholders. These assumptions were tested by means of a survey of principals in primary and secondary schools in these countries (n = 2239). The data analysis followed a three-step approach: (1) confirmatory factor analyses, (2) path modelling and (3) fitting of multiple-indicator multiple-cause models. The results indicate that Inspectorates of Education that use a differentiated model (in addition to regular visits), in which they evaluate both educational practices and outcomes of schools and publicly report inspection findings of individual schools, are the most effective. These changes seem to be mediated by improvements in the schools’ self-evaluations and the schools’ stakeholders’ awareness of the findings in the public inspection reports. However, differentiated inspections also lead to unintended consequences as principals report on narrowing the curriculum and on discouraging teachers from experimenting with new teaching methods

    Ireland, Norway and Turkey (vol 32, pg 395, 2020)

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    The original version of this article unfortunately contained a mistake. © 2020, Springer Nature B.V

    The unintended consequences of school inspection: the prevalence of inspection side-effects in Austria, the Czech Republic, England, Ireland, the Netherlands, Sweden, and Switzerland

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    It has been widely documented that accountability systems, including school inspections, bring with them unintended side effects. These unintended effects are often negative and have the potential to undo the intended positive effects. However the empirical evidence is limited. Through a European comparative study we have had the rare opportunity to collect empirical evidence and study the effects (both intended and unintended) of school inspections (a key system of accountability) in a systematic way, across seven countries. We present the findings of the unintended effects in this paper. Survey self-report responses from school principals in each country, with differing school inspection systems, are analysed to measure the prevalence of these unintended effects and to investigate the part played by pressure to do well in inspections. A key finding is that increasing pressure in school inspection systems is associated with the undesired effect of the narrowing and refocusing of the curriculum and instructional strategies. We also show that a proportion of school principals admit to misrepresenting the school in data sent to the inspectorate and show evidence for formalisation/proceduralisation (excessive focus on records) and ossification (fear of experimentation in teaching), although these factors are less related to changes in pressure
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