Vergleichende Analyse von Mausmodellen für die hereditären spastischen Paraplegien SPG31 und SPG72

Die Hereditären spastischen Paraplegien sind eine heterogene Familie von Erkrankungen, die durch eine Degeneration der Axone von Motoneuronen gekennzeichnet ist und letztlich zur spastischen Lähmung insbesondere der unteren Gliedmaßen führt. Ursächlich für die Subform SPG31 sind Mutationen in REEP1, die die dritthäufigste Ursache einer autosomal dominant vererbten HSP darstellen. REEP Proteine gelten als formgebende Proteine des ER. Mutationen in REEP2, einem paralogen Protein derselben Unterfamilie der REEP/DP1/Yop1p-Proteinfamilie, wurden als Auslöser der SPG72 identifiziert. Im Rahmen dieser Arbeit wurde Reep2 in der Maus ausgeschaltet, um mithilfe dieses Tiermodells Rückschlüsse auf die Pathophysiologie der Erkrankung ziehen zu können. Zum Vergleich wurden auch unveröffentlichte Daten des bereits publizierten murinen SPG31 Modells erneut ausgewertet und vergleichende in vitro Untersuchungen durchgeführt. Als histopathologisches Korrelat für eine progrediente Gangstörung konnte ein Verlust von Axonen mit großem Durchmesser in verschiedenen Arealen des lumbalen Rückenmarks nachgewiesen werden. Nach ultrastrukturellen Auswertungen der Purkinje-Zellen von Weibchen im Alter von zwei Jahren konnten auch dort signifikante Veränderungen beobachtet werden. Auffällig waren hierbei Veränderungen von ER Strukturen, Mitochondrien, Dictyosomen und lipid droplets. In vitro Untersuchungen an Überexpressionskonstrukten von Wildtyp REEP1 und REEP2 sowie einiger Deletionsmutanten konnten die Lokalisierung an ER und an Mitochondrien belegen. REEP1 besitzt darüber hinaus die Fähigkeit, direkt an lipid droplets zu binden. Zudem wurde auch eine erhöhte Anfälligkeit für die Bildung von toxischen Aggregaten des REEP1 Proteins beobachtet. Eine weitere Besonderheit von REEP1 ist eine muskelspezifische Isoform, welche aufgrund eines zusätzlichen Miniexons eine stärkere Ähnlichkeit zu den übrigen REEP2-4 Proteinen besitzt

Incentive for a health-oriented school development. The school development award "Gute gesunde Schule" in North Rhine-Westphalia

Der Schulentwicklungspreis Gute gesunde Schule hat zum Ziel, Schulen für ihre gesundheitsorientierte Schulentwicklungsarbeit auszuzeichnen. Es wird über die Grundlagen und Ergebnisse der Evaluationen berichtet. Hinsichtlich der Teilnahmemotivation zeigt sich, dass vor allem der Bedarf, Impulse für die Schulentwicklung zu erhalten, noch vor der Möglichkeit externer Rückmeldungen, dem Preisgeld und der öffentlichen Auszeichnung, eine bedeutende Rolle spielt. Zudem können Rückschlüsse für die Priorisierung von Qualitätsbereichen für eine gesundheitsorientierte Schulentwicklung gezogen werden. (DIPF/Orig.)The aim of the school development award "Gute gesunde Schule" ["Good and Healthy School"] is to reward schools for their health oriented organizational development. The procedure and main evaluation results are reported. The results show that the main motivation to participate is the necessity to receive impulses for school development. Furthermore, external feedback, prize money, and public recognition play a significant role for participation. The data also permit to infer a prioritization of quality aspects which may be used as a strategy for health oriented school development. (DIPF/Orig.

Empowering Consumers to Make Environmentally Sustainable Online Shopping Decisions: A Digital Nudging Approach

An ever-increasing share of people is using online shopping to satisfy their consumer needs. This has led to a vivid discussion regarding the environmental sustainability of e-commerce that also emphasized the role that consumer's decisions can play in mitigating its negative impacts. However, while many individuals state that they are willing to act more sustainably, they often struggle to follow through with their `green' intentions. We propose digital nudging as an approach to encourage environmentally sustainable online shopping decisions and empower consumers to act in line with their intentions. In an online experiment with 323 participants, we evaluate the effectiveness of three different nudging interventions (defaults, active choice, and self-nudging) to promote environmentally sustainable shipping options in an online store and assess the consumers' ethics and empowerment perceptions of the nudges. We find that all nudges are effective in changing decisions, but default nudges lead to negative perceptions among consumers

Changes in the spot shape of a small-radius electron beam during deviation on a horizontal surface in the pressure range of 10-30 pascales

The results of investigation about electron beam size changes during deviation of the beam on a horizontal surface in the pressure range of 10-30 Pa are presented. It is shown, that the size of the beam on longitudinal projection of the deviation increases monotonically, on the perpendicular - the beam size remains unchanged. Influence of helium pressure in the range of 10-30 Pa on the beam diameter is not depended

Concept for the cost prognosis in the industrialization of highly iteratively developed physical products

With the ongoing technological progress and increasing global competition, companies are facing a continuously changing market environment. Due to the volatility of the market, rapid product adjustments and shorter product life cycles are required. Changing customer requirements are rarely taken into account, leading to inventions that do not make the transition to innovations. Highly iterative product development poses a possibility to integrate the customer voice into the development process and thus shorten the time-to-market and enable companies to respond to changes in requirements. Within the scope of highly iterative product development methods, cost analysis remains one of the main challenges for companies. Since the scope of development is not known at the beginning of a project, neither development nor industrialization costs can be specified. This, however, is essential for product and process development to meet cost-related customer requirements and for forecasting the production and investment budgets. With existing methods, it is either possible to agree to a fixed development budget and target price or to enable the customer to make changes during development. The concept presented in this paper aims to counteract this challenge. Therefore, existing approaches are analyzed with regard to derived requirements for the transfer from highly iterative and integrated product and process development to agile cost analysis. Influencing factors on product and production process costs are identified based on findings from literature. By aligning the influencing factors and requirements, dependencies between target costs of a product and degrees of freedom of highly iterative product and production process development can be derived and used for the development of a framework for iterative cost analysis. In conclusion, a concept for an agile cost prognosis for the industrialization of highly iterative developed physical products is presented

Network platform for sound information analyzis

A new technique of audio information processing for automatic analysis and classification of records, including speech, has been proposed. The technique is based on information representation in the form of associative semantic (cognitive) network structure and deals with amplitude and frequency layers both

Oxidative destruction of organic pollutants on modified polypropylene fiber

The paper shows the prospects of using composites based on polypropylene fiber with embedded particles of metal Fe{0} and Al{0}. Samples were used for oxidative destruction of organic compounds under conditions of the Fenton-like system and visible radiation

How Can Physical Activity Be Promoted Among Children and Adolescents? A Systematic Review of Reviews Across Settings

Introduction: A vast majority of children and adolescents are physically inactive. As a result, high obesity rates and related diseases have made physical activity promotion a politically relevant topic. In order to form the basis for political decision making, evidence is required regarding the efficacy and effectiveness of interventions for physical activity promotion. In contrast to previous research, this systematic review of reviews targets three key settings (family and home, childcare, school), and is among the largest to have been conducted. Methods: A systematic review of reviews was conducted as part of a large-scale project to develop national recommendations for physical activity promotion in Germany. Six electronic databases were searched and inclusion criteria were defined. Two independent reviewers screened the titles and abstracts of potentially relevant literature. 213 reviews were identified and categorised by target group. A total of 74 reviews were identified dealing with children and adolescents. Each review underwent a quality assessment. Results: 39 reviews with the highest quality and relevance were analysed. Three reviews focused on the family and home setting, 4 on the childcare setting, 28 on the school setting and 4 on other settings. Evidence revealed the key role played by parents in promoting physical activity in children within each setting. Furthermore, evidence pointed toward the efficacy of multi-component interventions in the childcare and school setting. Several evidence-based intervention strategies were identified for childcare facilities and schools. Discussion: The review of reviews identified a number of promising strategies for PA promotion among children and adolescents. Among reviews, multi-component interventions in childcare facilities and schools stand out prominently. At the same time, the review of reviews indicated that there is still a lack of studies on the efficacy of interventions that go beyond the individual level. We recommend that future research should also target community and policy level interventions and interventions other than the school setting. In order to make more specific recommendations regarding the scale-up of promising intervention strategies, further knowledge about the effectiveness, health equity and cost effectiveness of interventions is needed