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The Federal Ministry of Labour and Social Affairs (Bundesministerium für Arbeit und Soziales, BMA) is supporting 73 projects in Germany using European Union (EU) funds in the amount of € 26 million. By providing the subsidies, the European Commission and the German Federal Government are hoping to implement Corporate Social Responsibility (CSR) among German small and medium-sized businesses (SMBs). The project run by Bonn-Rhein-Sieg University is one of these CSR projects. It is aimed at providing comprehensive information on CSR to the businesses in question and at emphasizing their responsibility along the supply chain.
Web-based Editor for YAWL
(2013)
This paper presents a web-based editor that offers YAWL editing capabilities and comprehensive support for the XML format of YAWL. The open-source project Signavio Core Components is extended with a graphical user interface (GUI) for parts of the YAWL Language, and an import-/export component that converts between YAWL and the internal format of Signavio Core Components. This conversion, between the web-based editor and the offcial YAWL Editor, is lossless so both tools may be used together. Compared to the offcial YAWL Editor, the web-based editor is missing some features, but could still facilitate the usage of the YAWL system in use cases that are not supported by a desktop application.
Computers will soon be powerful enough to simulate consciousness. The artificial life community should start to try to understand how consciousness could be simulated. The proposal is to build an artificial life system in which consciousness might be able to evolve. The idea is to develop internet-wide artificial universe in which the agents can evolve. Users play games by defining agents that form communities. The communities have to perform tasks, or compete, or whatever the specific game demands. The demands should be such that agents that are more aware of their universe are more likely to succeed. The agents reproduce and evolve within their user’s machine, but can also sometimes transfer to other machine across the internet. Users will be able to choose the capabilities of their agents from a fixed list, but may also write their own powers for their agents.
Botnets
(2013)
Malware poses one of the major threats to all currently operated computer systems. The scale of the problem becomes obvious by looking at the global economic loss caused by different kinds of malware, which is estimated to be more than US$ 10 billion every year. Botnets, a special kind of malware, are used to reap economic gains by criminals as well as for politically motivated activities. In contrast to other kinds of malware, botnets utilize a hidden communication channel to receive commands from their operator and communicate their current status. The ability to execute almost arbitrary commands on the infected machines makes botnets a general-purpose tool to perform malicious cyber-activities. (Verlagsangaben)
Die Jahresberichte der Hochschule Bonn-Rhein-Sieg haben jedes Mal ein anderes Schwerpunktthema. Für den Jahresbericht 2012 lautet das Thema "Verantwortlich handeln und Vorbild sein: Die Hochschule in der Gesellschaft".
Den Anfang bildet ein Gespräch zwischen dem Intendanten der Deutschen Welle, Erik Bettermann, und Hochschulpräsident Hartmut Ihne über Verantwortung in der Ausbildung und das Engagement in der Entwicklungszusammenarbeit. In den Kapiteln Studium & Lehre, Forschung, Campus, Region und Internationales findet sich ein vielfältiges Themenspektrum, wobei die Übergänge fließend sind, denn viele Themen ließen sich auch durchaus anderen Kapiteln zuordnen.
Sonderseiten sind in der neuen Ausgabe der "Pause" gewidmet: Forschungssemestern und Sabbatjahr, die Pause im wissenschaftlichen Fokus oder die ganz normale Kaffeepause. Pausen müssen sein.
The Report starts with an interview between Eric Bettermann, Director of the German radio station Deutsche Welle, and University President Hartmut Ihne, which deals with responsibility in education and our University’s activities in the area of development cooperation. The chapters “Studies & Research”, “Research”, “Campus” , “The Region and International Issues” cover a wide spectrum of topics that are not rigidly defined because many topics might just as readily be assigned to other chapters.
In the latest edition, some special pages have been dedicated to the topic of “Taking a break”, i.e. to research semesters and sabbaticals, to breaks as a scientific focal point or to absolutely normal coffee breaks. Breaks are an essential part of our lives.
Förderpreise 2013
(2013)
Für kleinere Unternehmen mit geringen Ressourcen ist die Gestaltung des QM-Systems eine beträchtliche Herausforderung: Welche Methoden und Maßnahmen sind nötig und bestgeeignet, um die Qualitätskosten nachhaltig zu senken? Durch individuelle und ganzheitliche Betrachtung des Unternehmens sowie Einsatz der Kraftfeldanalyse gelang es einem Metallverarbeiter, ein maßgeschneidertes und dauerhaft wirksames QM-System zu implementieren.
Corporate Social Responsibility ist freiwillig, aber keineswegs beliebig. Um sich als CSR-Unternehmen zu qualifizieren, muss ein systematisches und geplantes Engagement als nachhaltiges Unternehmen nachgewiesen und auch dokumentiert werden. Dies wird auch für Unternehmen der Immobilienwirtschaft zunehmend wichtiger, weil die Anforderungen vonseiten der Stakeholder der Unternehmen wachsen. Die Analyse zeigt, dass die deutschen Immobilienunternehmen im internationalen Vergleich gut dastehen. Ihr Anteil an allen nach der Global Reporting Initiative berichtenden Immobilienunternehmen lag im Jahr 2012 bei 15 Prozent. Von den betrachteten 135 Unternehmen in Deutschland klassifiziert sich jedoch nur ein kleiner Teil als CSR-Unternehmen. Durch eine bessere Dokumentation des Engagements kann die Anzahl an Unternehmen rasch vergrößert werden.
The criteria for assessing the quality of rubber materials are the polymer or copolymer composition and the additives. These additives include plasticizers, extender oils, carbon black, inorganic fillers, antioxidants, heat and light stabilizers, processing aids, cross-linking agents, accelerators, retarders, adhesives, pigments, smoke and flame retardants, and others. Determination of additives in polymers or copolymers generally requires the extraction of these substances from the matrix as a first step, which can be challenging, and the subsequent analysis of the extracted additives by gas chromatography (GC), GC-mass spectrometry (MS), high performance liquid chromatography (HPLC), HPLC-MS, capillary electrophoresis, thin-layer chromatography, and other analytical techniques. In the present work, nitrile rubber materials were studied using direct analytical flash pyrolysis hyphenated to GC and electrospray ionization MS in both scan and selected ion monitoring modes to demonstrate that this technique is a good tool to identify the organic additives in nitrile rubber.
YAWL Symposium 2013. Proceedings of the First YAWL Symposium, Sankt Augustin, Germany, June 7, 2013
(2013)
Als Basis für Simulationen innerhalb virtueller Umgebungen werden meist unterliegende Semantiken benötigt. Im Fall von Verkehrssimulationen werden in der Regel definierte Verkehrsnetzwerke genutzt. Die Erstellung dieser Netzwerke wird meist per Hand durchgeführt, wodurch sie fehleranfällig ist und viel Zeit erfordert. Dieses Projekt wurde im Rahmen des AVeSi Projektes durchgeführt, in dem an der Entwicklung einer realistischen Verkehrssimulation für virtuelle Umgebung geforscht wird. Der im Projekt angestrebte Simulationsansatz basiert auf der Nutzung von zwei Komplexitätsebenen – einer mikroskopischen und einer mesoskopischen. Um einen Übergang zwischen den Simulationsebenen zu realisieren ist eine Verknüpfung der Verkehrsnetzwerke notwendig, was ebenfalls mit einem hohen Zeitaufwand verbunden ist. In diesem Bericht werden Modelle für Verkehrsnetzwerke beider Ebenen vorgestellt. Anschließend wird ein Ansatz beschrieben, der eine automatische Generierung und Verknüpfung von Verkehrsnetzwerken beider Modelle ermöglicht. Als Grundlage für die Generierung der Netzwerke dienen Daten im OpenDRIVE®-Format. Zur Evaluierung wurden wirklichkeitsgetreue OpenStreetMap-Daten, durch Verwendung einer Drittanbietersoftware, in OpenDRIVE®-Datensätze überführt. Es konnte nachgewiesen werden, dass es durch den Ansatz möglich ist, innerhalb weniger Minuten, große Verkehrsnetzwerke zu erzeugen, auf denen unmittelbar Simulationen ausgeführt werden können. Die Qualität der zur Evaluierung generierten Netzwerke reicht jedoch für Umgebungen, in denen ein hoher Realitätsgrad gefordert wird, nicht aus, was einen zusätzlichen Bearbeitungsschritt notwendig macht. Die Qualitätsprobleme konnten darauf zurückgeführt werden, dass der Detailgrad, der den Evaluierungsdaten zu Grunde liegenden OpenStreetMap-Daten, nicht hoch genug und der Überführungsprozess nicht ausreichend transparent ist.
Produktionswirtschaft
(2013)
Annual Report 2011 - 2012
(2013)
Vom Signal zur Information
(2013)
"Die richtige Information zur richtigen Zeit am richtigen Ort", so lautet das Diktum der modernen Informationsgesellschaften. Methoden zur Beschaffung aktueller Informationen lernen die Studierenden des Fachbereichs Sozialversicherung gleich in mehreren Modulen während ihres Studiums in Hennef. Auf die Nutzung von BG-internen Informationssystemen wird dabei ebenso eingegangen wie auf die Inanspruchnahme sogenannter Informationsprovider wie beispielsweise dem Deutschen Institut für Medizinische Dokumentation und Information (DIMDI) oder den National Institutes of Health (NIH).
Der „vertrackte“ § 14 SGB IX
(2013)
Real-Time Simulation of Camera Errors and Their Effect on Some Basic Robotic Vision Algorithms
(2013)
The BRICS component model: a model-based development paradigm for complex robotics software systems
(2013)
Power train models are required to simulate hence predict energy consumption of vehicles. Efficiencies for different components in power train are required. Common procedures use digitalised shell models (or maps) to model the efficiency of Internal Combustion Engines (ICE) and manual gearboxes (MG). Errors are connected with these models and affect the accuracy of the calculation. The accuracy depends on the configuration of the simulation, the digitalisation of the data and the data used. This paper evaluates these sources of error. The understanding of the source of error can improve the results of the modelling by more than eight percent.
This work extends the affordance-inspired robot control architecture introduced in the MACS project [35] and especially its approach to integrate symbolic planning systems given in [24] by providing methods to automated abstraction of affordances to high-level operators. It discusses how symbolic planning instances can be generated automatically based on these operators and introduces an instantiation method to execute the resulting plans. Preconditions and effects of agent behaviour are learned and represented in Gärdenfors conceptual spaces framework. Its notion of similarity is used to group behaviours to abstract operators based on the affordance-inspired, function-centred view on the environment. Ways on how the capabilities of conceptual spaces to map subsymbolic to symbolic representations to generate PDDL planning domains including affordance-based operators are discussed. During plan execution, affordance-based operators are instantiated by agent behaviour based on the situation directly before its execution. The current situation is compared to past ones and the behaviour that has been most successful in the past is applied. Execution failures can be repaired by action substitution. The concept of using contexts to dynamically change dimension salience as introduced by Gärdenfors is realized by using techniques from the field of feature selection. The approach is evaluated using a 3D simulation environment and implementations of several object manipulation behaviours.
Molecular modeling is an important subdomain in the field of computational modeling, regarding both scientific and industrial applications. This is because computer simulations on a molecular level are a virtuous instrument to study the impact of microscopic on macroscopic phenomena. Accurate molecular models are indispensable for such simulations in order to predict physical target observables, like density, pressure, diffusion coefficients or energetic properties, quantitatively over a wide range of temperatures. Thereby, molecular interactions are described mathematically by force fields. The mathematical description includes parameters for both intramolecular and intermolecular interactions. While intramolecular force field parameters can be determined by quantum mechanics, the parameterization of the intermolecular part is often tedious. Recently, an empirical procedure, based on the minimization of a loss function between simulated and experimental physical properties, was published by the authors. Thereby, efficient gradient-based numerical optimization algorithms were used. However, empirical force field optimization is inhibited by the two following central issues appearing in molecular simulations: firstly, they are extremely time-consuming, even on modern and high-performance computer clusters, and secondly, simulation data is affected by statistical noise. The latter provokes the fact that an accurate computation of gradients or Hessians is nearly impossible close to a local or global minimum, mainly because the loss function is flat. Therefore, the question arises of whether to apply a derivative-free method approximating the loss function by an appropriate model function. In this paper, a new Sparse Grid-based Optimization Workflow (SpaGrOW) is presented, which accomplishes this task robustly and, at the same time, keeps the number of time-consuming simulations relatively small. This is achieved by an efficient sampling procedure for the approximation based on sparse grids, which is described in full detail: in order to counteract the fact that sparse grids are fully occupied on their boundaries, a mathematical transformation is applied to generate homogeneous Dirichlet boundary conditions. As the main drawback of sparse grids methods is the assumption that the function to be modeled exhibits certain smoothness properties, it has to be approximated by smooth functions first. Radial basis functions turned out to be very suitable to solve this task. The smoothing procedure and the subsequent interpolation on sparse grids are performed within sufficiently large compact trust regions of the parameter space. It is shown and explained how the combination of the three ingredients leads to a new efficient derivative-free algorithm, which has the additional advantage that it is capable of reducing the overall number of simulations by a factor of about two in comparison to gradient-based optimization methods. At the same time, the robustness with respect to statistical noise is maintained. This assertion is proven by both theoretical considerations and practical evaluations for molecular simulations on chemical example substances.
Realism and plausibility of computer controlled entities in entertainment software have been enhanced by adding both static personalities and dynamic emotions. Here a generic model is introduced which allows the transfer of findings from real-life personality studies to a computational model. This information is used for decision making. The introduction of dynamic event-based emotions enables adaptive behavior patterns. The advantages of this new model have been validated with a four-way crossroad in a traffic simulation. Driving agents using the introduced model enhanced by dynamics were compared to agents based on static personality profiles and simple rule-based behavior. It has been shown that adding an adaptive dynamic factor to agents improves perceivable plausibility and realism. It also supports coping with extreme situations in a fair and understandable way.
This paper examines how students learn to collaborate in English by participating in an intercultural project that focuses on teaching students to work together on a digital writing project using various online tools, and participated in this digital collaboration project. Mixed groups of students, two French and two German, used several synchronous and asynchronous tools to communicate with their counterparts (Facebook, WordPress blog, WIMS e-learning platform, email, videoconferencing). Students had to produce an article together, comparing French and German attitudes about a topic they negotiated freely in their groups. Before publishing their post, students were expected to peer-review the article written by their group. Once published, the stage consisted of voting for the best posts on the e-learning platform, WIMS. A videoconference was also organized to create cohesion between the participants. The result of the student evaluations, together with the administrative, technical vastly differing university setups is presented.
Qualitätsverbesserung und Zeitersparnis bei der Stipendienvergabe durch automatisierten Workflow
(2013)
Für die Vergabe der Deutschlandstipendien hatte die Hochschule anfangs ein Verfahren festgelegt, das viel manuelle Arbeitsschritte umfasst: Die Studierenden hatten ihre Bewerbungsunterlagen schriftlich einzureichen. Dazu gehörten neben einem Motivationsschreiben, einem Ausdruck des aktuellen Notenspiegels alle weiteren Referenzen zur Einschätzung der Bewerbung gemäß den gesetzlichen Auswahlkriterien. Als Grundlage zur Bewertung der „sozialen Kriterien“ sollten die Bewerberinnen und Bewerber ein Gutachten eines Professors oder einer Professorin der Hochschule einholen.
Earth’s nearest candidate supermassive black hole lies at the centre of the Milky Way1. Its electromagnetic emission is thought to be powered by radiatively inefficient accretion of gas from its environment2, which is a standard mode of energy supply for most galactic nuclei. X-ray measurements have already resolved a tenuous hot gas component from which the black hole can be fed3. The magnetization of the gas, however, which is a crucial parameter determining the structure of the accretion flow, remains unknown. Strong magnetic fields can influence the dynamics of accretion, remove angular momentum from the infalling gas4, expel matter through relativistic jets5 and lead to synchrotron emission such as that previously observed6, 7, 8. Here we report multi-frequency radio measurements of a newly discovered pulsar close to the Galactic Centre9, 10, 11, 12 and show that the pulsar’s unusually large Faraday rotation (the rotation of the plane of polarization of the emission in the presence of an external magnetic field) indicates that there is a dynamically important magnetic field near the black hole. If this field is accreted down to the event horizon it provides enough magnetic flux to explain the observed emission—from radio to X-ray wavelengths—from the black hole.
Although most individuals who gamble do so without any adverse consequences, some individuals develop a recurrent, maladaptive pattern of gambling behaviour, often called pathological gambling or gambling disorder, that is associated with financial losses, disruption of family and interpersonal relationships, and co-occurring psychiatric disorders. Identifying whether different types of gambling modalities vary in their ability to lead to maladaptive patterns of gambling behaviour is essential to develop public policies that seek to balance access to gambling opportunities with minimizing risk for the potential adverse consequences of gambling behaviour. Until recently, assessing the risk potential of different types of gambling products was nearly impossible. ASTERIG, initially developed in Germany in 2006-2010, is an assessment tool to measure and to evaluate the risk potential of any gambling product based on scores on ten dimensions. In doing so, it also allows a comparison to be drawn between the addictive potential of different gambling products. Furthermore, the tool highlights where the specific risk potential of each specific gambling product lies. This makes it a valuable tool at the legislative, case law, and administrative levels as it allows the risk potential of individual gambling products to be identified and to be compared globally and across 10 different dimensions of risk potential. We note that specific gambling products should always be evaluated rather than product groups (lotteries, slot machines) or providers, as there may be variations among those product groups that impact their risk potential. For example, slot machines may vary on the amount of jackpot, which may influence their risk potential.
Distributed systems comprise distributed computing systems, distributed information systems, and distributed pervasive systems. They are often very complex and their implementation is challenging. Intensive and continuous testing is indispensable to ensure reliability and high quality of a distributed system. The testing process should have a high degree of automation, not only on lower levels (i.e. unit and module testing), but also on higher testing levels (e.g. system, integration, and acceptance tests). To achieve automation on higher testing levels virtual infrastructure components (e.g. virtual machines, virtual networks) that are offered as a Service (IaaS) can be employed. The elasticity of on-demand computation resources fits well together with the varying resource demands of automated test execution.
A methodology for automated acceptance testing of distributed systems that uses virtual infrastructure is presented. It is founded on a task-oriented model that is used to abstract concurrency and asynchronous, remote communication in distributed systems. The model is used as groundwork for a domain-specific language that allows expressing tests for distributed systems in the form of scenarios. On the one hand, test scenarios are executable and, therefore, fully automated. On the other hand, test scenarios represent requirements to the system under test making an automated, example-based verification possible.
A prototypical implementation is used to apply the developed methodology in the context of two different case studies. The first case study uses RCE as an example of a distributed, workflow-driven integration environment for scientific computing. The second one uses MongoDB as an example of a document-oriented database system that offers distributed data storage through master-slave replication. The results of the experimental evaluation indicate that the developed acceptance testing methodology is a useful approach to design, build, and execute tests for distributed systems with high quality and a high degree of automation.
Updating a shared data structure in a parallel program is usually done with some sort of high-level synchronization operation to ensure correctness and consistency. However, underlying synchronization instructions in a processor architecture are costly and rather limited in their scalability on larger multi-core/multi-processors systems. In this paper, we examine work queue operations where such costly atomic update operations are replaced with non-atomic modifiers (simple read+write). In this approach, we trade the exact amount of work with atomic operations against doing more and redundant work but without atomic operations and without violating the correctness of the algorithm. We show results for the application of this idea to the concrete scenario of parallel Breadth First Search (BFS) algorithms for undirected graphs on two large NUMA shared memory system with up to 64 cores.
Angewandte Makroökonomie
(2013)
Makroökonomische Ereignisse wie die Schuldenkrise, Rezession, Arbeitslosigkeit und Inflation haben nicht nur gesamtwirtschaftliche Konsequenzen, sondern auch vielfältige Berührungspunkte zum täglichen Leben. Diese Ereignisse sind häufig komplex und für den Einzelnen nicht immer leicht zu durchschauen. Um Studierende auf die globalen Herausforderungen von Wirtschaft, Gesellschaft und Umwelt vorzubereiten ist in diesem Lehrbuch explizit auch das Thema der nachhaltigen Entwicklung integriert. Außerdem werden die großen Themen der Makroökonomie teilweise gebündelt behandelt, um die vielfältigen Zusammenhänge zwischen den einzelnen Gebieten transparenter zu gestalten. Dies hat für Studierende und Lehrende u.a. den Vorteil, dass eine modulare Verwendung möglich ist.
Information reliability and automatic computation are two important aspects that are continuously pushing the Web to be more semantic. Information uploaded to the Web should be reusable and extractable automatically to other applications, platforms, etc. Several tools exist to explicitly markup Web content. The Web services may also have a positive role on the automatic processing of Web contents, especially when they act as flexible and agile agents. However, Web services themselves should be developed with semantics in mind. They should include and provide structured information to facilitate their use, reuse, composition, query, etc. In this chapter, the authors focus on evaluating state-of-the-art semantic aspects and approaches in Web services. Ultimately, this contributes to the goal of Web knowledge management, execution, and transfer.
Tamoxifen therapy of invasive breast cancer has been associated with increased levels of endothelin-1 (ET-1) so that an endothelin-1 receptor (ETR) blockade has been suggested as a new therapeutic approach. This study analyzed the relationship between Tamoxifen and ET-1 signalling in invasive breast cancer. Using paraffinized tissue from 50 randomly chosen cases of invasive and in-situ ductal carcinoma from our archive, the expression of ETRs was analyzed by immune histology. ETRs were regularly detectable in normal breast tissue, but rarely in adjacent tumor areas (3/50 cases). By immunoprecipitation, a complex was found consisting of ET-1, estrogen receptors and Tamoxifen. Consequently, transcription of several target genes of ET-1 and estrogen receptors was detectable (interleukin-6, wnt-11 and a vimentin spliceform). In particular, the combination of Tamoxifen, ET-1, and estrogen receptors induced further increasing levels of these target genes. Some of these genes have been found upregulated in metastatically spreading breast cancer cells. We conclude: i) ETRs do not play a role in invasive or in-situ ductal breast cancer; ii) estrogen receptors and Tamoxifen build a complex with ET-1; and iii) upregulated transcription of target genes by ET-1–estrogen receptor–Tamoxifen complex may negatively influence breast cancer prognosis. These results indicate a role for ET-1 in Tamoxifen treated breast cancer patients leading to a potentially worsening prognosis.
Increased endothelin-1 decreases PKC alpha (PKCα), resulting in high miRNA 15a levels in kidney tumors. Breast cancer cells treated with ET-1, β-estrogen, Tamoxifen, Tamoxifen + β-estrogen and Tamoxifen + ET-1 were analysed regarding miRNA 15a expression. Significantly increased miRNA 15a levels were found after ET-1, becoming further increased in Tamoxifen + ET-1 treated cells. Our group already showed that miRNA 15a induces MAPK p38 splicing resulting in a truncated product called Mxi-2, whose function has yet to be defined in tumors. We described for the first time in ET-1 induced tumor cells that Mxi-2 builds a complex with Ago2, a miRNA binding protein, which is important for the localization of miRNAs to the 3′UTR of target genes. Furthermore, we show that Mxi-2/Ago2 is important for the interaction with the miRNA 1285 which binds to the 3′end of the tumor suppressor gene p53, being responsible for the downregulation of p53. Tissue arrays from breast cancer patients were performed, analysing Mxi-2, p53 and PKCα. Since the Mxi-2 levels increase in Tamoxifen + ET-1 treated cells, we claim that increasing ET-1 levels in Tamoxifen treated breast cancer patients are responsible for decreasing p53 levels. In summary, ET-1 decreases nuclear PKCα levels, while increasing the amount of miRNA 15a. This causes high levels of Mxi-2, necessary for complex formation with Ago2. The newly identified Mxi-2/Ago2 complex interacting with miRNA 1285 leads to increased 3′UTR p53 interaction, resulting in decreased p53 levels and subsequent tumor progression. This newly identified mechanism is a possible explanation for the development of ET-1 induced tumors.
More than 25 years ago, it was a big surprise for physiologists that nitric oxide (NO) was identified as the endothelium derived relaxing factor which is responsible for endothelium-induced smooth muscle relaxation (Ignarro et al., 1987). Until then, small gaseous molecules were simply regarded as byproducts of cellular metabolism which were unlikely to be of any physiological relevance. The discovery that NO was synthesized by specific enzymes (NO-synthases), upon stimulation by specific, physiologically relevant stimuli (e.g., acetylcholine stimulation of endothelial cells), as well as the fact that it acted on specific cellular targets (e.g., soluble guanylate cyclase), set the course for numerous studies which investigated the physiological roles of gaseous signaling molecules—in other words, gasotransmitters (Wang, 2002).
Studierende und Absolventen der neuen Bachelorstudiengänge berichten über ihre Erfahrungen im Praxissemester. Sie wollen damit anderen Studierenden Mut machen, ihren persönlichen Weg ins Ausland zu finden, den ersten Schritt zu wagen und vor allem zur Planung eines eigenen Auslandspraxissemesters anregen. Ihre Beiträge zeigen, persönliche Erfahrungen und Entdeckungen im Ausland können das Leben bereichern und Freundschaften über Kulturen und Länder hinweg entstehen lassen.
Ornithine transcarbamylase (OTC) deficiency is the most common urea cycle defect. The clinical presentation in female manifesting carriers varies both in onset and severity. We report on a female with insulin dependent diabetes mellitus and recurrent episodes of hyperammonemia. Since OTC activity measured in a liver biopsy sample was within normal limits, OTC deficiency was initially excluded from the differential diagnoses of hyperammonemia. Due to moderately elevated homocitrulline excretion, hyperornithinemia-hyperammonemia-homocitrullinuria-syndrome was suggested, but further assays in fibroblasts showed normal ornithine utilization. Later, when mutation analysis of the OTC gene became available, a known pathogenic missense mutation (c.533C>T) in exon 5 leading to an exchange of threonine-178 by methionine (p.Thr178Met) was detected. Skewed X-inactivation was demonstrated in leukocyte DNA. In the further clinical course the girl developed marked obesity. By initiating physical activities twice a week, therapeutic control of both diabetes and OTC deficiency improved, but obesity persisted. In conclusion, our case confirms that normal hepatic OTC enzyme activity measured in a single liver biopsy sample does not exclude a clinical relevant mosaic of OTC deficiency because of skewed X-inactivation. Mutation analysis of the OTC gene in whole blood may be a simple way to establish the diagnosis of OTC deficiency. The joint occurrence of OTC deficiency and diabetes in a patient has not been reported before.
Die vorliegende Geldwäsche-Studie soll im deutschen Glücksspielmarkt soll die Diskussion über die Geldwäschemöglichkeiten im Bereich Online Poker versachlichen und grundsätzliche Präventionsmaßnahmen herleiten. Auf dieser Grundlage wird die TÜV TRUST IT GmbH Unternehmensgruppe TÜV AUSTRIA als Auftraggeber der Studie mess- und bewertbare Prüfkriterien entwickeln und diese in ein Prüf- und Zertifizierverfahren überleiten. Damit werden dem Markt dann erstmals sachlich nachvollziehbare, wissenschaftlich fundiert, standardisierte Kriterien zur Verfügung stehen, um das Thema sachlich zu behandeln und Teilnehmer am Markt in die Lage zu versetzen, klare Regeln zu definieren und einzuhalten.
BACKGROUND
Hyperlysinemia is an autosomal recessive inborn error of L-lysine degradation. To date only one causal mutation in the AASS gene encoding α-aminoadipic semialdehyde synthase has been reported. We aimed to better define the genetic basis of hyperlysinemia.
METHODS
We collected the clinical, biochemical and molecular data in a cohort of 8 hyperlysinemia patients with distinct neurological features.
RESULTS
We found novel causal mutations in AASS in all affected individuals, including 4 missense mutations, 2 deletions and 1 duplication. In two patients originating from one family, the hyperlysinemia was caused by a contiguous gene deletion syndrome affecting AASS and PTPRZ1.
CONCLUSIONS
Hyperlysinemia is caused by mutations in AASS. As hyperlysinemia is generally considered a benign metabolic variant, the more severe neurological disease course in two patients with a contiguous deletion syndrome may be explained by the additional loss of PTPRZ1. Our findings illustrate the importance of detailed biochemical and genetic studies in any hyperlysinemia patient.
The Forum Nachhaltigkeit im Lebensmittelhandel (NIL; Sustainability in Food Retailing) is a collection of scientific papers dealing with various aspects of sustainability in the area of food retail trade.