challenges and institutional arrangements

Adaptation to climate change has become an integral part of climate change policies across the world. Based on the limited literature on the governance of climate change adaptation, the paper first highlights four key challenges governments face in this context, i.e. (i) how to better integrate adaptation policies horizontally across policy sectors and (ii) vertically across levels of government, (iii) how to integrate knowledge in adaptation policy decisions, and (iv) how to involve stakeholders in adaptation decisions. The paper then shows how selected OECD countries address these challenges when developing and implementing adaptation policies and instruments. We identify the most important governance mechanisms on the national level which constitute a distinct governance structure in each surveyed country, and highlight their objectives and specific foci on one or more governance challenges. The paper analyses dominant modes of emerging interaction patterns in the respective governance arrangements.Draft pape

Lokale Agenda 21 in Helsingborg und Wien - Stärken und Schwächen unterschiedlicher Organisationsstrukturen

"Die Lokale Agenda 21 (LA21) hat ihre Wurzeln in der Umweltkonferenz der Vereinten Nationen 1992 von Rio und strebt nachhaltige Entwicklung auf kommunalem Niveau an. Der Artikel vergleicht LA21-Prozesse zweier Städte, Helsingborg in Schweden und Wien, und gibt Einblick in verschiedenartige Umsetzungen gleicher politischer Verpflichtungen. Die strukturellen Rahmenbedingungen und die Organisationsformen der Prozesse werden vergleichend analysiert. Der Schwerpunkt liegt - analog zu den programmatischen Vorgaben der LA21 - auf der Analyse der Kooperation aller betroffenen AkteurInnen, der Kommunikationsqualitäten und der Partizipationsformen innerhalb der Prozesse. Die Ergebnisse der Studie illustrieren zwei nahezu diametrale Organisationsformen lokaler Politikgestaltung, wobei der schwedische Prozess als eher technokratisch-expertInnengeleitet und das österreichische Fallbeispiel als partizipatorisch-bürgerInnenorientiert gelten können." (Autorenreferat)"Local Agenda 21 has its roots in the Rio Earth-summit of 1992 and aims for sustainable development at community level. This article compares two Local Agenda 21 processes in Helsingborg, Sweden, and Vienna, Austria, and offers insights for the implementation variability of the same political commitments. The different contexts and organisations are analysed comparatively. Local Agenda 21 emphasises the importance of inclusion and co-operation of all involved parties. The study, therefore, focuses on the quality of communication and process participation. Study results indicate two contrary strategies of sustainable development at community level. While the Helsingborg process can be described as technocratic and expert-led, the Austrian case qualifies as participatory and citizen-oriented. The study demonstrates the different strengths and weaknesses of these processes." (author's abstract

PPA1 Deficiency Causes a Deranged Galactose Metabolism Recognizable in Neonatal Screening

Two siblings showed increased galactose and galactose-related metabolites in neonatal screening. Diagnostic workup did not reveal abnormalities in any of the known disease-causing enzymes involved in galactose metabolism. Using whole-exome sequencing, we identified a homozygous missense variant in PPA1 encoding the cytosolic pyrophosphatase 1 (PPA1), c.557C>T (p.Thr186Ile). The enzyme activity of PPA1 was determined using a colorimetric assay, and the protein content was visualized via western blotting in skin fibroblasts from one of the affected individuals. The galactolytic activity of the affected fibroblasts was determined by measuring extracellular acidification with a Seahorse XFe96 analyzer. PPA1 activity decreased to 22% of that of controls in the cytosolic fraction of homogenates from patient fibroblasts. PPA1 protein content decreased by 50% according to western blot analysis, indicating a reduced stability of the variant protein. The extracellular acidification rate was reduced in patient fibroblasts when galactose was used as a substrate. Untargeted metabolomics of blood samples revealed an elevation of other metabolites related to pyrophosphate metabolism. Besides hyperbilirubinemia in the neonatal period in one child, both children were clinically unremarkable at the ages of 3 and 14 years, respectively. We hypothesize that the observed metabolic derangement is a possible mild manifestation of PPA1 deficiency. Unresolved abnormalities in galactosemia screening might result in the identification of more individuals with PPA1 deficiency, a newly discovered inborn metabolic disorder (IMD)

Transforming European Water Governance? Participation and River Basin Management under the EU Water Framework Directive in 13 Member States

The European Union (EU) Water Framework Directive (WFD) requires EU member states to produce and implement river basin management plans, which are to be designed and updated via participatory processes that inform, consult with, and actively involve all interested stakeholders. The assumption of the European Commission is that stakeholder participation, and institutional adaptation and procedural innovation to facilitate it, are essential to the effectiveness of river basin planning and, ultimately, the environmental impact of the Directive. We analyzed official documents and the WFD literature to compare implementation of the Directive in EU member states in the initial WFD planning phase (2000–2009). Examining the development of participatory approaches to river basin management planning, we consider the extent of transformation in EU water governance over the period. Employing a mixed quantitative and qualitative approach, we map the implementation “trajectories” of 13 member states, and then provide a detailed examination of shifts in river basin planning and participation in four member states (Germany, Sweden, Poland and France) to illustrate the diversity of institutional approaches observed. We identify a general tendency towards increased, yet circumscribed, stakeholder participation in river basin management in the member states examined, alongside clear continuities in terms of their respective pre-WFD institutional and procedural arrangements. Overall, the WFD has driven a highly uneven shift to river basin-level planning among the member states, and instigated a range of efforts to institutionalize stakeholder involvement—often through the establishment of advisory groups to bring organized stakeholders into the planning process

Vokabellernen mit der Schlüsselwortmethode im Französischunterricht

Die vorliegende Diplomarbeit beschäftigt sich in theoretischer und praktischer Hinsicht eingehend mit der Schlüsselwortmethode (SWM), einer nachweislich effektiven Mnemotechnik, die speziell zum Lernen fremdsprachlicher Vokabeln eingesetzt werden kann. Aus dem vielfältigen am Buch- und Onlinemarkt vorhandenen populärwissenschaftlichen Selbstlernmaterial wurden drei aktuelle deutschsprachige Medien ausgewählt, die auf dem Prinzip der SWM basieren und speziell für das Lernen französischer Vokabeln konzipiert wurden – die beiden Sprachlernratgeber Wasch die Kuh. Mit Wortbildern hundert und mehr Französischvokabeln pro Stunde lernen von Geisselhart und Lange und Vokabel Lernen mit Eselsbrücken. Lernen mit der Schlüsselwortmethode. Grundwortschatz Französisch/Deutsch von Lindo sowie der Vokabellernkurs Französisch Grundwortschatz der Lernsoftware Memrise, welcher als App- und als Desktop-Version verfügbar ist. Zunächst wurden eine qualitative Inhaltsanalyse der drei Medien nach Mayring sowie auch kleinere Frequenzanalysen im Sinne der quantitativen Inhaltsanalyse durchgeführt, um den jeweiligen inhaltlichen und didaktischen Anspruch der Medien rekonstruieren zu können. Anschließend wurde in einer kritischen literaturgestützten Analyse das didaktische Potential der SWM vor dem Hintergrund des modernen kommunikativen Fremdsprachenunterrichts herausgearbeitet. In weiterer Folge wurde aufgezeigt, wie sich die SWM zweckmäßig in den Französischunterricht einbeziehen lässt und welche Rolle dabei die drei untersuchten Medien spielen können. Eine wichtige Erkenntnis ist, dass die drei Medien partiell zwar sprachliche, didaktische und lexikographische Mängel aufweisen, ausgewählte Teile jedoch im Rahmen eines Vokabelstrategietrainings zur SWM angemessen einbezogen werden können. Insbesondere dem Lernprogramm Memrise kann aufgrund der lebensweltlichen Relevanz von Apps und Smartphones für Jugendliche sowie der Möglichkeit systematischen und zugleich spielerisch-motivierenden Übens von Vokabelgleichungen durchaus Potential für die unterrichtliche Wortschatzarbeit zugesprochen werden.The present diploma thesis deals with both the theoretical and practical aspects of the keyword method (KWM), a scientifically proven mnemonic device that is particularly effective in foreign language vocabulary learning. Three German media based on the KWM principle and specifically designed for French vocabulary acquisition are analysed – two self-help books, Wasch die Kuh. MitWortbildern hundert und mehr Französischvokabeln pro Stunde lernen by Geisselhart and Lange, Vokabel Lernen mit Eselsbrücken. Lernen mit der Schlüsselwortmethode. Grundwortschatz Französisch/Deutsch by Lindo and a vocabulary course named Französisch Grundwortschatz available as an app and desktop version within the educational software Memrise. First, a qualitative content analysis of the three media as well as minor quantitative frequency analysis were conducted with the aim to reconstruct the content-related and didactical quality of the media. Next, a critical literature-based analysis attempted to outline the didactical potential of the KWM for foreign language teaching and present possible ways of how to appropriately integrate the KWM in French classes considering the role the three media can play in that effort. One key finding is that each of the media shows particular linguistic, lexicographic and didactical deficiencies, yet all include certain parts that can successfully be exploited within vocabulary strategy training for the KWM. In particular, the program Memrise has considerable potential for vocabulary work in class when one considers the importance of apps and smartphones for today's youth and the opportunity to practice vocabulary in a systematic, gamified and thus motivating way

Machiavellian otium

E' online dal 10 dicembre 2023 un sito internet/database online sul tema dell'ozio nelle lettere di Niccolò Machiavelli realizzato/curato da una équipe di ricercatori e docenti internazionale, formata da Andrea Guidi, Stefano Saracino, Christian Feichtinger e Judith Frömmer. Non si tratta di mero ozio letterario, bensì di una concezione di 'otium' in senso molto ampio e con risvolti di una certa importanza per lo sviluppo del pensiero politico machiavelliano e in generale per una visione politica di certe pratiche sociali e culturali

PPA1 Deficiency Causes a Deranged Galactose Metabolism Recognizable in Neonatal Screening

Two siblings showed increased galactose and galactose-related metabolites in neonatal screening. Diagnostic workup did not reveal abnormalities in any of the known disease-causing enzymes involved in galactose metabolism. Using whole-exome sequencing, we identified a homozygous missense variant in PPA1 encoding the cytosolic pyrophosphatase 1 (PPA1), c.557C>T (p.Thr186Ile). The enzyme activity of PPA1 was determined using a colorimetric assay, and the protein content was visualized via western blotting in skin fibroblasts from one of the affected individuals. The galactolytic activity of the affected fibroblasts was determined by measuring extracellular acidification with a Seahorse XFe96 analyzer. PPA1 activity decreased to 22% of that of controls in the cytosolic fraction of homogenates from patient fibroblasts. PPA1 protein content decreased by 50% according to western blot analysis, indicating a reduced stability of the variant protein. The extracellular acidification rate was reduced in patient fibroblasts when galactose was used as a substrate. Untargeted metabolomics of blood samples revealed an elevation of other metabolites related to pyrophosphate metabolism. Besides hyperbilirubinemia in the neonatal period in one child, both children were clinically unremarkable at the ages of 3 and 14 years, respectively. We hypothesize that the observed metabolic derangement is a possible mild manifestation of PPA1 deficiency. Unresolved abnormalities in galactosemia screening might result in the identification of more individuals with PPA1 deficiency, a newly discovered inborn metabolic disorder (IMD)

Cytoplasmic location of NR4A1 in aggressive lymphomas is associated with a favourable cancer specific survival

Abstract The nuclear orphan receptor NR4A1 functions as tumour suppressor in aggressive lymphomas by pro-apoptotic genomic and non-genomic effects. Here, we immunohistochemically studied the clinico-pathological relevance of NR4A1 protein expression patterns in a cohort of 60 diffuse large B cell lymphoma (DLBCL) patients and non-neoplastic lymph nodes. We observed a significant association between high cytoplasmic NR4A1 and favourable cancer-specific survival and the germinal centre B cell-like subtype, respectively. Moreover, the percentage of lymphoma cells exhibiting cytoplasmic NR4A1 significantly correlated to those showing cleaved caspase 3. Complementary, functional profiling using gene set enrichment of Reactome pathways based on publicly available microarray data was applied to determine pathways potentially implicated in cytoplasmic localization of NR4A1 and validated by means of semi quantitative real-time PCR. The pathway analysis revealed changes in the ERK1/2 pathway, and this was corroborated by the finding that high cytoplasmic NR4A1 was associated with higher expression of ERK1/2 targets in our cohort. These data indicate that high cytoplasmic NR4A1 is associated with a favourable lymphoma-specific survival and highlights the importance of NR4A1 expression patterns as potential prognostic marker for risk assessment in aggressive lymphomas

Transforming European Water Governance? Participation and River Basin Management under the EU Water Framework Directive in 13 Member States

The European Union (EU) Water Framework Directive (WFD) requires EU member states to produce and implement river basin management plans, which are to be designed and updated via participatory processes that inform, consult with, and actively involve all interested stakeholders. The assumption of the European Commission is that stakeholder participation, and institutional adaptation and procedural innovation to facilitate it, are essential to the effectiveness of river basin planning and, ultimately, the environmental impact of the Directive. We analyzed oficial documents and the WFD literature to compare implementation of the Directive in EU membre states in the initial WFD planning phase (2000-2009). Examining the development of participatory approaches to river basin management planning, we consider the extent of transformation in EU water governance over the period. Employing a mixed quantitative and qualitative approach, we map the implementation "trajectories" of 13 member states, and then provide a detailed examination of shifts in river basin planning and participation in four member states (Germany, Sweden, Poland and France) to illustrate the diversity of institutional approaches observed. We identify a general tendency towards increased, yet circumscribed, stakeholder participation in river basin management in the member states examined, alongside clear continuities in terms of their respective pre-WFD institutional and procedural arrangements. Overall, the WFD has driven a highly uneven shift to river basin-level planning among the member states, and instigated a range of efforts to institutionalize stakeholder involvement-often through the establishment of advisory groups to bring organized stakeholders into the planning process

PSMC3 proteasome subunit variants are associated with neurodevelopmental delay and type I interferon production

A critical step in preserving protein homeostasis is the recognition, binding, unfolding, and translocation of protein substrates by six AAA-ATPase proteasome subunits (ATPase-associated with various cellular activities) termed PSMC1-6, which are required for degradation of proteins by 26 S proteasomes. Here, we identified 15 de novo missense variants in the PSMC3 gene encoding the AAA-ATPase proteasome subunit PSMC3/Rpt5 in 23 unrelated heterozygous patients with an autosomal dominant form of neurodevelopmental delay and intellectual disability. Expression of PSMC3 variants in mouse neuronal cultures led to altered dendrite development, and deletion of the PSMC3 fly ortholog Rpt5 impaired reversal learning capabilities in fruit flies. Structural modeling as well as proteomic and transcriptomic analyses of T cells derived from patients with PSMC3 variants implicated the PSMC3 variants in proteasome dysfunction through disruption of substrate translocation, induction of proteotoxic stress, and alterations in proteins controlling developmental and innate immune programs. The proteostatic perturbations in T cells from patients with PSMC3 variants correlated with a dysregulation in type I interferon (IFN) signaling in these T cells, which could be blocked by inhibition of the intracellular stress sensor protein kinase R (PKR). These results suggest that proteotoxic stress activated PKR in patient-derived T cells, resulting in a type I IFN response. The potential relationship among proteosome dysfunction, type I IFN production, and neurodevelopment suggests new directions in our understanding of pathogenesis in some neurodevelopmental disorders