The German Energy transition at the local level - A Discourse Network Analysis for identifying fostering and hindering discourse patterns and network structures

The German Energiewende is an enormous and far-reaching project, consisting of a huge variety of goals that aim in transiting Germany to a reduced and greener energy consumption. While the far-reaching goals are set by the federal government, achieving the goals is crucially dependent on their implementation at the local level. Empirically a huge variation in the implementation of this goals at the local level can be observed. This variation can only partly be explained by independent preconditions of the state (such as average income, size or land-use). This paper studies more subtle varying factors, that might have an influence on the successful implementation of renewable energies. Based on the agenda setting approach and the Advocacy Coalition Framework, this paper analyzes the relation between discourses, local policy network structures and the success of a region in implementing renewable energies. Therefor a Discourse Network Analysis (DNA) is conducted which identities differences in discourse contents and discourse network structures in four counties (two successful and two unsuccessful). Results support the hypotheses that the discourse content is strongly dependent on whether a county is rural or urban. Furthermore results show that the discourse network structures of successful counties tend to become more open and consensual over time, while in unsuccessful counties the discourse network structures become more clustered. This is a strong indicator for the connection between discourses, their network structures and the success of the successful implementation of the energy transition

Applying the energy efficiency first principle based on a decision-tree framework

Energy Efficiency First (EEF) is an established principle for European Union (EU) energy policy design. It highlights the exploitation of demand-side resources and prioritizes cost-effective options from the demand-side over other options from a societal cost-benefit perspective. However, the involvement of multiple decision-makers makes it difficult to implement. Therefore, we propose a flexible decision-tree framework for applying the EEF principle based on a review of relevant areas and examples. In summary, this paper contributes to applying the EEF principle by defining and distinguishing different types of cases - (1) policy-making, and (2) system planning and investment - identifying the most common elements, and proposing a decision-tree framework that can be flexibly constructed based on the elements for different cases. Finally, we exemplify the application of this framework with two example cases: (1) planning for demand-response in the power sector, and (2) planning for a district heating system

Current-driven switching of exchange biased spin-valve giant magnetoresistive nanopillars using a conducting nanoprobe

An array of exchange biased spin-valve giant-magnetoresistance nanopillars was fabricated and the current I dependence of the resistance R was investigated using an electrically conducting atomic-force microscope (AFM) probe contact at room temperature. We observed current induced switching in a MnIr/CoFe/Cu/CoFe/NiFe nanopillar using the AFM probe contact. Current-driven switching using nanoprobe contact is a powerful method for developing nonvolatile and rewritable magnetic memory with high density

Social innovation supports inclusive and accelerated energy transitions with appropriate governance

Accelerating energy transitions that are both sustainable and just remains an important challenge, and social innovation can have a key role in this transition. Here, we examine the diversity and potential of social innovation in energy systems transformation, synthesizing original mixed methods data from expert interviews, document analysis, social innovation experiments, a representative survey, and an expert survey. Based on a thematic analysis of these data, we advance four key findings: (1) the diversity of social innovation in energy is best understood when recognizing core social practices (thinking, doing, and organizing) and accounting for changes in social relations (cooperation, exchange, competition, and conflict); (2) governance, policy networks, and national context strongly shape social innovation dynamics; (3) processes of social innovation are implicated by multidimensional power relations that can result in transformative changes; and (4) social innovation in energy generally has strong social acceptance among citizens, benefits local communities and is legitimized in key community and city organizations. We discuss an agenda for 9 future research directions on social innovation in energy, and conclude with insights related to national context, governance, and acceleration

Genetic landscape of pediatric acute liver failure of indeterminate origin.

BACKGROUND AIMS Pediatric acute liver failure (PALF) is a life-threatening condition. In Europe, main causes are viral infections (12-16%) and inherited metabolic diseases (14-28%). Yet, in up to 50% of cases the underlying etiology remains elusive, challenging clinical management, including liver transplantation. We systematically studied indeterminate PALF cases referred for genetic evaluation by whole-exome sequencing (WES), and analyzed phenotypic and biochemical markers, and the diagnostic yield of WES in this condition. METHODS With this international, multicenter observational study, patients (0-18 y) with indeterminate PALF were analyzed by WES. Data on the clinical and biochemical phenotype were retrieved and systematically analyzed. RESULTS In total, 260 indeterminate PALF patients from 19 countries were recruited between 2011 and 2022, of whom 59 had recurrent PALF (RALF). WES established a genetic diagnosis in 37% of cases (97/260). Diagnostic yield was highest in children with PALF in the first year of life (46%), and in children with RALF (64%). Thirty-six distinct disease genes were identified. Defects in NBAS (n=20), MPV17 (n=8) and DGUOK (n=7) were the most frequent findings. When categorizing, most frequent were mitochondrial diseases (45%), disorders of vesicular trafficking (28%) and cytosolic aminoacyl-tRNA synthetase deficiencies (10%). One-third of patients had a fatal outcome. Fifty-six patients received liver transplants. CONCLUSION This study elucidates a large contribution of genetic causes in PALF of indeterminate origin with an increasing spectrum of disease entities. The high proportion of diagnosed cases and potential treatment implications argue for exome or in future rapid genome sequencing in PALF diagnostics

Genetic landscape of pediatric acute liver failure of indeterminate origin

BACKGROUND AND AIMS: Pediatric acute liver failure (PALF) is a life-threatening condition. In Europe, the main causes are viral infections (12%-16%) and inherited metabolic diseases (14%-28%). Yet, in up to 50% of cases the underlying etiology remains elusive, challenging clinical management, including liver transplantation. We systematically studied indeterminate PALF cases referred for genetic evaluation by whole-exome sequencing (WES), and analyzed phenotypic and biochemical markers, and the diagnostic yield of WES in this condition. APPROACH AND RESULTS: With this international, multicenter observational study, patients (0-18 y) with indeterminate PALF were analyzed by WES. Data on the clinical and biochemical phenotype were retrieved and systematically analyzed. RESULTS: In total, 260 indeterminate PALF patients from 19 countries were recruited between 2011 and 2022, of whom 59 had recurrent PALF. WES established a genetic diagnosis in 37% of cases (97/260). Diagnostic yield was highest in children with PALF in the first year of life (41%), and in children with recurrent acute liver failure (64%). Thirty-six distinct disease genes were identified. Defects in NBAS (n=20), MPV17 (n=8), and DGUOK (n=7) were the most frequent findings. When categorizing, the most frequent were mitochondrial diseases (45%), disorders of vesicular trafficking (28%), and cytosolic aminoacyl-tRNA synthetase deficiencies (10%). One-third of patients had a fatal outcome. Fifty-six patients received liver transplantation. CONCLUSIONS: This study elucidates a large contribution of genetic causes in PALF of indeterminate origin with an increasing spectrum of disease entities. The high proportion of diagnosed cases and potential treatment implications argue for exome or in future rapid genome sequencing in PALF diagnostics

Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study

Background Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure. Methods We generated a progression score on the basis of principal component analysis of prospectively acquired longitudinal changes in motor, cognitive, and imaging measures in the 218 indivduals in the TRACK-HD cohort of Huntington's disease gene mutation carriers (data collected 2008–11). We generated a parallel progression score using data from 1773 previously genotyped participants from the European Huntington's Disease Network REGISTRY study of Huntington's disease mutation carriers (data collected 2003–13). We did a genome-wide association analyses in terms of progression for 216 TRACK-HD participants and 1773 REGISTRY participants, then a meta-analysis of these results was undertaken. Findings Longitudinal motor, cognitive, and imaging scores were correlated with each other in TRACK-HD participants, justifying use of a single, cross-domain measure of disease progression in both studies. The TRACK-HD and REGISTRY progression measures were correlated with each other (r=0·674), and with age at onset (TRACK-HD, r=0·315; REGISTRY, r=0·234). The meta-analysis of progression in TRACK-HD and REGISTRY gave a genome-wide significant signal (p=1·12 × 10−10) on chromosome 5 spanning three genes: MSH3, DHFR, and MTRNR2L2. The genes in this locus were associated with progression in TRACK-HD (MSH3 p=2·94 × 10−8 DHFR p=8·37 × 10−7 MTRNR2L2 p=2·15 × 10−9) and to a lesser extent in REGISTRY (MSH3 p=9·36 × 10−4 DHFR p=8·45 × 10−4 MTRNR2L2 p=1·20 × 10−3). The lead single nucleotide polymorphism (SNP) in TRACK-HD (rs557874766) was genome-wide significant in the meta-analysis (p=1·58 × 10−8), and encodes an aminoacid change (Pro67Ala) in MSH3. In TRACK-HD, each copy of the minor allele at this SNP was associated with a 0·4 units per year (95% CI 0·16–0·66) reduction in the rate of change of the Unified Huntington's Disease Rating Scale (UHDRS) Total Motor Score, and a reduction of 0·12 units per year (95% CI 0·06–0·18) in the rate of change of UHDRS Total Functional Capacity score. These associations remained significant after adjusting for age of onset. Interpretation The multidomain progression measure in TRACK-HD was associated with a functional variant that was genome-wide significant in our meta-analysis. The association in only 216 participants implies that the progression measure is a sensitive reflection of disease burden, that the effect size at this locus is large, or both. Knockout of Msh3 reduces somatic expansion in Huntington's disease mouse models, suggesting this mechanism as an area for future therapeutic investigation

Local Policy Networks : Actors, Challenges and Cooperation in the Case of the German Energy Transition

The German Energiewende is a project of national concern. The term subsumes Germany's efforts to transform the energy system towards a green energy supply. Moreover, it represents the German response to major national and global challenges, and aims to sustain the challenged German forerunner position in supporting green technologies. Major policy problems that are addressed with this far-reaching project are the global climate change, the corresponding need to reduce greenhouse gas emissions, as well as Germany's over dependence on foreign energy resources, and the wish to opt out of nuclear energy. This thesis follows current research findings, in arguing that urban as well as rural areas are decisive for a successful transition process and moreover, the renunciation from fossil fuels, nationally as well as globally. An orchestration of the opportunities and challenges that both types of areas face, will be crucial for an affordable, secure, and sustainable energy provision. Rural counties as well as urban areas vary distinctively in the extent to which they address sustainability, climate change, and energy related challenges. This thesis applies a relational perspective to add to the evolving body of literature which analyzes what determines the distinct local energy policy-making processes and outcomes. Therefore, this thesis combines the encompassing policy subsystem view of the Advocacy Coalition Framework with the study of policy networks, and understands policy-making as the interplay of diverse actors who collaborate in order to shape policy outcomes around a certain policy problem in their favor. This thesis aims to describe, characterize, and compare local energy policy subsystems, focusing on three central elements of policy subsystems: Actors, problems, and collaboration. A mixed-methods design is applied in order to study regional similarities and differences in who participates in the process, which policy problems are (perceived as) salient, and how discourses and collaboration evolve over time. Data was collected through a Discourse Network Analysis as well as with an online survey, questioning actors actively engaged in local energy policy-making. A comparative research design was chosen in order to identify similarities and differences between urban and rural areas as well as between those counties that are more and those counties that are less successful in implementing the energy transition. The results show that differences between urban and rural areas exist, concerning the composition of actors and their presence in the discourse as well as regarding the policy issues that are perceived to be salient. Furthermore, the results indicate that the development of network structures differs between successful and less successful counties, and that belief similarity and perceived agreement are drivers of collaboration within local energy policy networks.publishe

Studying the impacts of digitalization and further societal trends on energy demand

Digitalization has the possibility to fundamentally change the current ways things are done. From the digitalization in private households (as for example voice control systems, smart thermostats and streaming operators), to digitalization in the transport sector (autonomous electric cars with smart loading infrastructure), to increasingly digitalized processes in the industry which can be highly optimized through automation