From policy to identity: regions at the heart of the European project

The thesis presents a comparative analysis of the scope and objectives of four EU regions’ European policies and programmes. It evaluates the extent to which regions’ European engagement is targeted to achieving regional economic development on the one hand and European social integration and identity construction on the other hand. The analysis starts with a comparative evaluation of the four case study regions’ European policies and is substantiated by the findings of over 60 interviews with regional political elites and civil servants in the four case study regions: Germany’s Brandenburg; Belgium’s Wallonia; France’s Nord – Pas de Calais; and the South West of England. This thesis advances a more comprehensive understanding of regional governments as European actors, whether political elites and civil servants aim to promote European identity-building through their policies, as well as which regional characteristics further impact the scope and objectives of their European policies. This thesis provides evidence-based answers to the research question posed: What are the scope and objectives of regions’ European policies and what role does European identity play in them? The thesis research has grown out of the context of regions’ EU integration; the multi-level governance approach; the increase of regions’ political authority vis-à-vis European politics; and the uncertainty on whether regional political actors (political elites and civil servants) aim to foster a European identity. Research has not yet observed, compared and analysed the objectives of regions’ European policies in terms of European identity-building. This thesis research has taken an important step in pioneering this area of research by undertaking case studies in four EU regions

How CO2 Capture and Storage Can Mitigate Carbon Leakage

Most CO2 abatement policies reduce the demand for fossil fuels and therefore their price in international markets. If these policies are not global, this price decrease raises emissions in countries without CO2 abatement policies, generating “carbon leakage”. On the other hand, if the countries which abate CO2 emissions are net fossil fuel importers, they benefit from this price decrease, which reduces the abatement cost. In contrast, CO2 capture and storage (CCS) does not reduce fossil fuel demand, therefore it generates neither this type of leakage nor this negative feedback on abatement costs. We quantify these effects with the global hybrid general equilibrium model Imaclim-R and show that they are quantitatively important. Indeed, for a given unilateral abatement in OECD countries, leakage is more than halved in a scenario with CCS included among the abatement options, compared to a scenario prohibiting CCS. We show that the main reason for this difference in leakage is the above-mentioned international fossil fuel price feedback. This article does not intend to assess the desirability of CCS, which has many other pros and cons. It just identifies a consequence of CCS that should be taken into account, together with many others, when deciding to what extent CCS should be developed.CO2 Capture and Storage, Carbon Leakage

Decarbonizing development: three steps to a zero-carbon future

This report lays out three steps for a smooth transition to a zero-carbon future and provides data, examples and policy advice to help countries makes the shift. Overview Getting to zero net emissions and stabilizing climate change starts with planning for the long-term future and not stopping at short-term goals. It means getting prices right as part of a broad policy package that can trigger changes in both investments and behaviors, and it requires smoothing the transition for those most affected. A new World Bank report walks policymakers through those three steps with data, examples and policy advice to help put countries on a path to decarbonizing their development in a smooth and orderly way. The solutions exist, and they are affordable – if governments take action today, the report says

Clinical and molecular genetics of the multiple pterygium syndromes

The multiple pterygium syndromes are a heterogeneous group of conditions in which arthrogryposis (joint contractures), pterygia (webbing) and a variety of other developmental anomalies are present. It is caused by lack of fetal movement in the womb. Mutations in CHRNG, the embryonic subunit of the acetylcholine receptor (AChR), cause some of the cases. CHRNG mutation analysis was undertaken in a large patient cohort of 100 families and the mutations identified were included in a new Locus Specific Database. Genotype phenotype analysis showed that pterygia were almost invariably present in the CHRNG mutation positive patients. It was hypothesised that mutations in other genes necessary for fetal AChR function may cause fetal akinesia. Using a candidate gene approach a homozygous frameshift mutation in RAPSN was identified in one family and a homozygous splice site DOK7 mutation in second family. Mild mutations in both RAPSN and DOK7 have been previously identified in the congenital myasthenic syndromes (CMS). Thus, mild mutations in RAPSN and DOK7 cause CMS whereas severe mutations cause fetal akinesia. Finally, work was done to identify a novel cause of fetal akinesia in a consanguineous family using an autozygosity mapping approach. A region of homozygosity was located and candidate genes sequenced

On fitting planetary systems in counter-revolving configurations

In Gayon & Bois (2008) and Gayon etal (2009), (i) we studied the theoretical feasibility and efficiency of retrograde mean motion resonances (i.e. two planets are both in orbital resonance and in counter-revolving configuration), (ii) we showed that retrograde resonances can generate interesting mechanisms of stability, and (iii) we obtained a dynamical fit involving a counter-revolving configuration that is consistent with the observations of the HD73526 planetary system. In the present paper, we present and analyze data reductions assuming counter-revolving configurations for eight compact multi-planetary systems detected through the radial velocity method. In each case, we select the best fit leading to a dynamically stable solution. The resulting data reductions obtained in rms and chi values for counter-revolving configurations are of the same order, and sometimes slightly better, than for prograde configurations. In the end, these fits tend to show that, over the eight studied multi-planetary systems, six of them could be regulated by a mechanism involving a counter-revolving configuration.Comment: 4 pages, 1 figure, 2 tables, accepted for publication in MNRAS letters (August 10, 2009

Climate policies as a hedge against the uncertainty on future oil supply

International audienceDespite the inextricable link between oil scarcity and climate change, the interplay between these two issues is paradoxically an underworked area. This article uses a global energy-economy model to address the link between future oil supply and climate change and assesses in a common framework both the costs of climate policies and oil scarcity. It shows that, in the context of a limited and uncertain amount of ultimately recoverable oil resources, climate policies reduce the world vulnerability to peak oil. Climate policies, therefore, appear as a hedging strategy against the uncertainty on oil resources, in addition to their main aim of avoiding dangerous climate change. This co-benefit is estimated at the net present value of US$11,500 billion. Eventually, reducing the risk of future economic losses due to oil scarcity may appear as a significant side-benefit of climate policies to many decision-makers

A Recurrent Pathogenic Variant in TPM2 Reveals Further Phenotypic and Genetic Heterogeneity in Multiple Pterygium Syndrome-Related Disorders.

Multiple pterygium syndrome (MPS) disorders are a phenotypically and genetically heterogeneous group of conditions characterised by multiple joint contractures (arthrogryposis), pterygia (joint webbing) and other developmental defects. MPS is most frequently inherited in an autosomal recessive fashion but X-linked and autosomal dominant forms also occur. Advances in genomic technologies have identified many genetic causes of MPS-related disorders and genetic diagnosis requires large targeted next generation sequencing gene panels or genome-wide sequencing approaches. Using the Illumina TruSightOne clinical exome assay we identified a recurrent heterozygous missense substitution in TPM2 (encoding beta tropomyosin) in three unrelated individuals. This was confirmed to have arisen as a de novo event in the two patients with parental samples. TPM2 mutations have previously been described in association with a variety of dominantly inherited neuromuscular phenotypes including nemaline myopathy, congenital fibre-type disproportion, distal arthrogryposis and trismus pseudocamptodactyly, and in a patient with autosomal recessive Escobar syndrome and a nemaline myopathy. The three cases reported here had overlapping but variable features. Our findings expand the range of TMP2-related phenotypes and indicate that de novo TMP2 mutations should be considered in isolated cases of MPS-related conditions. This article is protected by copyright. All rights reserved

Landscape mosaics maps as a basis for spatial assessment and negotiation of ecosystem services and their trade-offs at the meso-scale: Examples from Laos, Madagascar and China

Mapping ecosystem services (ES) and their trade-offs is a key requirement for informed decision making for land use planning and management of natural resources that aim to move towards increasing the sustainability of landscapes. The negotiations of the purposes of landscapes and the services they should provide are difficult as there is an increasing number of stakeholders active at different levels with a variety of interests present on one particular landscape.Traditionally, land cover data is at the basis for mapping and spatial monitoring of ecosystem services. In light of complex landscapes it is however questionable whether land cover per se and as a spatial base unit is suitable for monitoring and management at the meso-scale. Often the characteristics of a landscape are defined by prevalence, composition and specific spatial and temporal patterns of different land cover types. The spatial delineation of shifting cultivation agriculture represents a prominent example of a land use system with its different land use intensities that requires alternative methodologies that go beyond the common remote sensing approaches of pixel-based land cover analysis due to the spatial and temporal dynamics of rotating cultivated and fallow fields.Against this background we advocate that adopting a landscape perspective to spatial planning and decision making offers new space for negotiation and collaboration, taking into account the needs of local resource users, and of the global community. For this purpose we introduce landscape mosaicsdefined as new spatial unit describing generalized land use types. Landscape mosaics have allowed us to chart different land use systems and land use intensities and permitted us to delineate changes in these land use systems based on changes of external claims on these landscapes. The underlying idea behindthe landscape mosaics is to use land cover data typically derived from remote sensing data and to analyse and classify spatial patterns of this land cover data using a moving window approach. We developed the landscape mosaics approach in tropical, forest dominated landscapesparticularly shifting cultivation areas and present examples ofour work from northern Laos, eastern Madagascarand Yunnan Province in China