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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).
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)
Fast täglich werden neue Angriffe auf IT-Systeme bekannt, bei denen sensible Daten entwendet werden. Das vorliegende Buch vermittelt die wesentlichen Grundlagen und Technologien, die zur Absicherung von Computernetzwerken benötigt werden. Stets legen die Autoren dabei Wert auf eine verständliche Darstellung, die – soweit möglich – auf abstrakte Modelle und formalen Notationen verzichtet. Zu jedem Kapitel werden Aufgaben zur Kontrolle von Wissensstand und Verständnis angeboten.
One idea behind Open Educational Resources (OERs) is opening up the access to learning resources for stakeholders who were not the originally targeted users. Even though making educational resources available for the public already is a remarkable achievement, their usefulness often is limited to a very particular context because of unclear or missing appropriateness regarding other contexts. In this paper, contextual appropriateness is investigated as a special quality criterion for OERs. We will introduce barriers against the use of OERs and demands from the educational community that need to be addressed in order to overcome such barriers. We will show that the hitherto implemented quality standards for Technology Enhanced Learning do not yet fully support such particular demands and discuss which additional steps are required for the context of OERs. We conclude with an outlook and recommendations that can open up the full potential of OERs.
Open Discovery Space
(2013)
The aim of our research is preserving the learners’ initial motivation in educational settings by avoiding unnecessary conflicts that could decrease the learners’ joy of learning. In order to get a better understanding of particularly cul-ture-related factors that could jeopardize the learners’ motivation in international e-Learning scenarios, we devel-oped and exemplarily implemented the standardized questionnaire ‘Learning Culture’ in the Higher Education contexts of Germany and South Korea. Regarding motivation, we analysed how the students evaluated their own motivational predispositions towards outer influences, their purpose of learning and affections towards particular knowledge, and their strategies to deal with educational tasks that appear unmanageable or too difficult for them.
A structural mapping of mutations causing succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency
(2013)
Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency is a rare inherited metabolic disorder of ketone metabolism, characterized by ketoacidotic episodes and often permanent ketosis. To date there are ~20 disease-associated alleles on the OXCT1 gene that encodes the mitochondrial enzyme SCOT. SCOT catalyzes the first, rate-limiting step of ketone body utilization in peripheral tissues, by transferring a CoA moiety from succinyl-CoA to form acetoacetyl-CoA, for entry into the tricarboxylic acid cycle for energy production. We have determined the crystal structure of human SCOT, providing a molecular understanding of the reported mutations based on their potential structural effects. An interactive version of this manuscript (which may contain additional mutations appended after acceptance of this manuscript) may be found on the web address:
http://www.thesgc.org/jimd/SCOT
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.
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.
BACKGROUND
Propionic acidemia is an inherited disorder caused by deficiency of propionyl-CoA carboxylase. Although it is one of the most frequent organic acidurias, information on the outcome of affected individuals is still limited.
STUDY DESIGN/METHODS
Clinical and outcome data of 55 patients with propionic acidemia from 16 European metabolic centers were evaluated retrospectively. 35 patients were diagnosed by selective metabolic screening while 20 patients were identified by newborn screening. Endocrine parameters and bone age were evaluated. In addition, IQ testing was performed and the patients' and their families' quality of life was assessed.
RESULTS
The vast majority of patients (>85%) presented with metabolic decompensation in the neonatal period. Asymptomatic individuals were the exception. About three quarters of the study population was mentally retarded, median IQ was 55. Apart from neurologic symptoms, complications comprised hematologic abnormalities, cardiac diseases, feeding problems and impaired growth. Most patients considered their quality of life high. However, according to the parents' point of view psychic problems were four times more common in propionic acidemia patients than in healthy controls.
CONCLUSION
Our data show that the outcome of propionic acidemia is still unfavourable, in spite of improved clinical management. Many patients develop long-term complications affecting different organ systems. Impairment of neurocognitive development is of special concern. Nevertheless, self-assessment of quality of life of the patients and their parents yielded rather positive results.
BACKGROUND
Metabolic control and dietary management of patients with phenylketonuria (PKU) are based on single blood samples obtained at variable intervals. Sampling conditions are often not well-specified and intermittent variation of phenylalanine concentrations between two measurements remains unknown. We determined phenylalanine and tyrosine concentrations in blood over 24 hours. Additionally, the impact of food intake and physical exercise on phenylalanine and tyrosine concentrations was examined. Subcutaneous microdialysis was evaluated as a tool for monitoring phenylalanine and tyrosine concentrations in PKU patients.
METHODS
Phenylalanine and tyrosine concentrations of eight adult patients with PKU were determined at 60 minute intervals in serum, dried blood and subcutaneous microdialysate and additionally every 30 minutes postprandially in subcutaneous microdialysate. During the study period of 24 hours individually tailored meals with defined phenylalanine and tyrosine contents were served at fixed times and 20 min bicycle-ergometry was performed.
RESULTS
Serum phenylalanine concentrations showed only minor variations while tyrosine concentrations varied significantly more over the 24-hour period. Food intake within the patients' individual diet had no consistent effect on the mean phenylalanine concentration but the tyrosine concentration increased up to 300% individually. Mean phenylalanine concentration remained stable after short-term bicycle-exercise whereas mean tyrosine concentration declined significantly. Phenylalanine and tyrosine concentrations in dried blood were significantly lower than serum concentrations. No close correlation has been found between serum and microdialysis fluid for phenylalanine and tyrosine concentrations.
CONCLUSIONS
Slight diurnal variation of phenylalanine concentrations in serum implicates that a single blood sample does reliably reflect the metabolic control in this group of adult patients. Phenylalanine concentrations determined by subcutaneous microdialysis do not correlate with the patients' phenylalanine concentrations in serum/blood.
Reactive oxygen species and the bacteriostatic and bactericidal effects of isoconazole nitrate
(2013)
Kommentierung zu § 39 BDSG
(2013)
In Software development, the always beta principle is used to successfully develop innovation based on early and continuous user feedback. In this paper we discuss how this principle could be adapted to the special needs of designing for the Smart Home, where we do not just take care of the software, but also release hardware components. In particular, because of the 'materiality' of the Smart Home one could not just make a beta version available on the web, but an essential part of the development process is also to visit the 'beta' users in their home, to build trust, to face the real world issues and provide assistance to make the Smart Home work for them. After presenting our case study, we will then discuss the challenges we faced and how we dealt with them.
The Java Virtual Machine (JVM) executes the compiled bytecode version of a Java program and acts as a layer between the program and the operating system. The JVM provides additional features such as Process, Thread, and Memory Management to manage the execution of these programs. The Garbage Collection (GC) is part of the memory management and has an impact on the overall runtime performance because it is responsible for removing dead objects from the heap. Currently, the execution of a program needs to be halted during every GC run. The problem of this stop-the-world approach is that all threads in the JVM need to be suspended. It would be desirable to have a thread-local GC that only blocks the current thread and does not affect any other threads. In particular, this would improve the execution of multi-threaded Java programs. An object that is accessible by more than one thread is called escaped. It is not possible to thread-locally determine if escaped objects are still alive so that they cannot be handled in a thread-local GC. To gain significant performance improvements with a thread-local GC, it is therefore necessary to determine if it is possible to reliably predict if a given object will escape. Experimental results show that the escaping of objects can be predicted with high accuracy based on the line of code the object was allocated from. A thread-local GC was developed to minimize the number of stop-the-world GCs. The prototype implementation delivers a proof-of-concept that shows that this goal can be achieved in certain scenarios.
PhD Project Management
(2013)