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Serine/threonine kinase 4 (STK4) deficiency is an autosomal recessive genetic condition that leads to primary immunodeficiency (PID) typically characterized by lymphopenia, recurrent infections and Epstein Barr Virus (EBV) induced lymphoproliferation and -lymphoma. State-of-the-art treatment regimens consist of prevention or treatment of infections, immunoglobulin substitution (IVIG) and restoration of the immune system by hematopoietic stem cell transplantation. Here, we report on two patients from two consanguineous families of Turkish (patient P1) and Moroccan (patient P2) decent, with PID due to homozygous STK4 mutations. P1 harbored a previously reported frameshift (c.1103 delT, p.M368RfsX2) and P2 a novel splice donor site mutation (P2; c.525+2 T>G). Both patients presented in childhood with recurrent infections, CD4 lymphopenia and dysregulated immunoglobulin levels. Patient P1 developed a highly malignant B cell lymphoma at the age of 10 years and a second, independent Hodgkin lymphoma 5 years later. To our knowledge she is the first STK4 deficient case reported who developed lymphoma in the absence of detectable EBV or other common viruses. Lymphoma development may be due to the lacking tumor suppressive function of STK4 or the perturbed immune surveillance due to the lack of CD4+ T cells. Our data should raise physicians' awareness of [1] lymphoma proneness of STK4 deficient patients even in the absence of EBV infection and [2] possibly underlying STK4 deficiency in pediatric patients with a history of recurrent infections, CD4 lymphopenia and lymphoma and unknown genetic make-up. Patient P2 experienced recurrent otitis in childhood, but when she presented at the age of 14, she showed clinical and immunological characteristics similar to patients suffering from Autoimmune Lymphoproliferative Syndrome (ALPS): elevated DNT cell number, non-malignant lymphadenopathy and hepatosplenomegaly, hematolytic anemia, hypergammaglobulinemia. Also patient P1 presented with ALPS-like features (lymphadenopathy, elevated DNT cell number and increased Vitamin B12 levels) and both were initially clinically diagnosed as ALPS-like. Closer examination of P2, however, revealed active EBV infection and genetic testing identified a novel STK4 mutation. None of the patients harbored typically ALPS-associated mutations of the Fas receptor mediated apoptotic pathway and Fas-mediated apoptosis was not affected. The presented case reports extend the clinical spectrum of STK4 deficiency.
Females are influenced more than males by visual cues during many spatial orientation tasks; but females rely more heavily on gravitational cues during visual-vestibular conflict. Are there gender biases in the relative contributions of vision, gravity and the internal representation of the body to the perception of upright? And might any such biases be affected by low gravity? 16 participants (8 female) viewed a highly polarized visual scene tilted ±112° while lying supine on the European Space Agency's short-arm human centrifuge. The centrifuge was rotated to simulate 24 logarithmically spaced g-levels along the long axis of the body (0.04-0.5g at ear-level). The perception of upright was measured using the Oriented Character Recognition Test (OCHART). OCHART uses the ambiguous symbol "p" shown in different orientations. Participants decided whether it was a "p" or a "d" from which the perceptual upright (PU) can be calculated for each visual/gravity combination. The relative contribution of vision, gravity and the internal representation of the body were then calculated. Experiments were repeated while upright. The relative contribution of vision on the PU was less in females compared to males (t=-18.48, p≤0.01). Females placed more emphasis on the gravity cue instead (f:28.4%, m:24.9%) while body weightings were constant (f:63.0%, m:63.2%). When upright (1g) in this and other studies (e.g., Barnett-Cowan et al. 2010, EJN, 31,1899) females placed more emphasis on vision in this task than males. The reduction in weight allocated by females to vision when in simulated low-gravity conditions compared to when upright under normal gravity may be related to similar female behaviour in response to other instances of visual-vestibular conflict. Why this is the case and at which point the perceptual change happens requires further research.
Um das digitale Storytelling für Medienunternehmen lukrativ nutzbar zu machen, existiert eine zunehmende Zahl von Tools, Software also, die das deutlich weniger zeitaufwendige Produzieren mithilfe zur Verfügung stehender Seitenvorlagen möglich machen. Drei oftmals verwendete Tools zur Produktion als auch zur Veröffentlichung von Beiträgen im digitalen Storytelling sind Atavist, Pageflow und Shorthand. Statt eigenem Programmieren können verschiedene multimediale Elemente in der Regel mit wenigen Mausklicks integriert werden. Nicolas Kaufmann beschäftigt sich in seiner Abschlussarbeit zum Bachelor of Science mit dem Thema "Digitales Storytelling - Eine Untersuchung zu Darstellungsformen, Nutzen und Tools".
In 2018, in the US alone, it is estimated that 268,670 people will be diagnosed with breast cancer, and that 41,400 will die from it. Since breast cancers often become resistant to therapies, and certain breast cancers lack therapeutic targets, new approaches are urgently required. A cell-stress response pathway, the unfolded protein response (UPR), has emerged as a promising target for the development of novel breast cancer treatments. This pathway is activated in response to a disturbance in endoplasmic reticulum (ER) homeostasis but has diverse physiological and disease-specific functions. In breast cancer, UPR signalling promotes a malignant phenotype and can confer tumours with resistance to widely used therapies. Here, we review several roles for UPR signalling in breast cancer, highlighting UPR-mediated therapy resistance and the potential for targeting the UPR alone or in combination with existing therapies.
Antioxidant activity is an essential feature required for oxygen-sensitive merchandise and goods, such as food and corresponding packaging as well as materials used in cosmetics and biomedicine. For example, vanillin, one of the most prominent antioxidants, is fabricated from lignin, the second most abundant natural polymer in the world. Antioxidant potential is primarily related to the termination of oxidation propagation reactions through hydrogen transfer. The application of technical lignin as a natural antioxidant has not yet been implemented in the industrial sector, mainly due to the complex heterogeneous structure and polydispersity of lignin. Thus, current research focuses on various isolation and purification strategies to improve the compatibility of lignin material with substrates and enhancing its stabilizing effect.
Amino acids perform multiple essential physiological roles in humans, and accordingly, their importance to health has been the subject of extensive attention. In this special issue of the Journal of Nutrition and Metabolism, we focus on the various inborn errors of amino acid metabolism, their diagnostic challenges, new treatment approaches, and recent advances in patient monitoring as well as clinical outcomes.
Here, we present a miR mechanism which is active in the nucleus and is essential for the production of intron included, C-terminal truncated and biologically active proteins, like e.g. Vim3. We exemplified this mechanism by miRs, miR-15a and miR-498, which are overexpressed in clear cell renal carcinoma or oncocytoma. Both miRs directly interact with DNA in an intronic region, leading to transcriptional stop, and therefore repress the full length version of the pre-mRNA, resulting in intron included truncated proteins (Mxi-2 and Vim3). A computational survey shows that this miR:DNA interactions mechanism may be generally involved in regulating the human transcriptome, with putative interaction sites in intronic regions for over 1000 genes. In this work, an entirely new mechanism is revealed how miRs can repress full length protein translation, resulting in C-terminal truncated proteins.
3-Hydroxy-3-methylglutaryl-coenzyme A lyase (HMGCL, HMGCL) deficiency is a rare inborn error of ketogenesis. Even if the ketogenic enzyme is fully disrupted, an elevated signal for the ketone body acetoacetic acid is a frequent observation in the analysis of urinary organic acids, at least if derivatization is performed by methylation. We provide an explanation for this phenomenon and trace it back to degradation of the derivatized 3-hydroxy-3-methylglutaric acid and high temperature of the injector of the gas chromatograph.
TREE Jahresbericht 2017
(2018)
Knapp fünf Jahre nach Gründung als Fachbereichsinstitut und zwei Jahre nach Verankerung als zentrale wissenschaftliche Einrichtung der Hochschule präsentieren wir - nicht ganz ohne Stolz - den ersten Jahresbericht des Instituts TREE. Er soll in seiner Breite als auch in seiner Tiefe die Stärken unserer gemeinschaftlichen Anstrengungen im Forschungsfeld der nachhaltigen Technologien aufzeigen: interdisziplinär, forschungsstark, nachwuchsfördernd und gesellschaftszugewandt. TREE ist weiterhin ein im Aufbruch begriffenes Institut, aber gerade das Jahr 2017 zeigt auch, dass wir uns schon in der Wissenschaftslandkarte einen Namen machen konnten: nach NaWETec konnte mit dem Themenkomplex "Effiziente Transportalternativen" ein zweiter Forschungsschwerpunkt drittmittelgefördert etabliert werden. Erste Promotionen im Rahmen des TREE konnten erfolgreich abgeschlossen und interessante Nachwuchswissenschaftler für "FHKarrierewege" gewonnen werden.
Conclusion
(2018)
There is a paradigm shift from traditional content-based education and training to competencybased and practice-oriented training. This shift has occurred because practice-oriented teaching has been found to produce a training outcome that is industry focused, generating the relevant occupational standards. Competency-based training program often comprises of modules broken into segments called learning outcomes. These learning outcomes are based on criteria set by industry and assessment is designed to ensure students become competent in their respective areas of specialization.
Course Profiles
(2018)
The curricula of all degree programs at H-BRS have many different practice-oriented activities and focus on hands-on learning. In labs and small classrooms (30–60 persons), students get a personalized learning environment which is complemented with many individual and group projects that foster collaborative work situations. There are several main areas that students learn from working with industry, local organizations or public institutions.
German African Universities Program (GAUP) is a partnership of three universities encompassing Hochschule Bonn-Rhein-Sieg, University of Applied Sciences in Germany, University of Nairobi in Kenya, and University of Cape Coast in Ghana. Every year, a number of students from each university are competitively selected to take part in the exchange program in a foreign country for three months in the sponsored project. I participated in the exchange program in Germany during the winter semester 2016/2017. The experience was excellent and the benefits cannot be underestimated. The objective was to integrate with the university community in Germany and learn first-hand from their teaching approaches and experiences by attending classes and interacting in- and outside the classroom. It provided an opportunity for cross-cultural learning, hence preparing us to live and work in different parts of the world. Besides the classroom experience, learning was reinforced by exposure tours in the industry including the Coal mine industrial complex in Essen (UNESCO world heritage since 2001) and Rheinbach.
The exchange program enables students to travel from their home countries to a partner university in the German-African University project. Students from the University of Nairobi in Kenya and University of Cape Coast in Ghana travel to the Hochschule Bonn-Rhein-Sieg University of Applied Sciences and stay for three months attending classes and participating in academic activities together with German students. Similarly, students from Hochschule Bonn-Rhein-Sieg, University of Applied Sciences, travel to either West or East Africa and are hosted for three months by universities participating in the project. The program enables Kenyan students to accustom themselves to the German way of life and student-centered learning and disciplines. The program integrates fieldwork into the learning activities making education both a skill-imparting and fun process.
The nature of the program was an exchange program between Hochschule Bonn-Rhein-Sieg, University of Applied Sciences and the University of Cape Coast. The program was advertised and we applied. We were shortlisted for interview and we were selected as the candidates for the exchange program. The program took a period of five months. We set off from Accra, Ghana to Germany on 7th September 2015, and returned to Ghana on 25th January 2016.
The exchange program was aimed at giving students an international exposure through teaching and intercultural communication and to also enhance the existing relationship among the partner schools. The program lasted for a period of six months from September 2016 to February 2017. The main part of the program was the International Management program which comprised of four courses. The program offered us an opportunity to travel to four European countries to broaden our academic and social network.
From September 2016 to February 2017, I did an internship at the University of Cape Coast, Ghana (UCC) as part of my studies in Business Administration at Hochschule Bonn-Rhein-Sieg, University of Applied Sciences, Germany (H-BRS). At H-BRS, an internship of five or six months (or, alternatively, one exchange semester) is an obligatory part of the curriculum so students get hands-on experience even before they enter the job market. My internship was also part of the intercontinental partnership between UCC and H-BRS, which has resulted in many different projects.
I had an opportunity to visit Germany in 2016/2017 during which period I was on an exchange staff program between the University of Nairobi, Hochschule Bonn-Rhein-Sieg, University of Applied Sciences, Germany, and the University of Cape Coast, Ghana. My visit took me to the city of Bonn where the University of Bonn-Rhein-Sieg is located in the suburban area in the cities of Sankt Augustin, Rheinbach, and Hennef. I was able to interact with faculty members and students. During this period, the discussion I had with faculty mainly focused on various programs offered by the university and how they have been able to interact and partner with the industry and create linkages with various firms in Germany. It emerged from our discussion that the development of the curriculum by the university depends on such partnerships.
Competency-Based Teaching Using Simulation Exercises: Evidence of the University of Cape Coast
(2018)
Tertiary institutions exist to train manpower to solve local, national, and international problems. Products from such institutions should not be a problem to countries as in the case of some Sub-Saharan African countries including Ghana which has a high level of graduate unemployment. Among the causes of the problem is the nature of teaching or the syllabus or the programs students pursue while in such institutions. The paper discusses one of the teaching strategies used to make a course relevant for a program and for the working world. In this course, students are introduced to practice-oriented learning through simulation exercises. The project activities specifically seek to assess the students’ understanding of business formation; examine students’ understanding of sustainability, creativity and innovation of business ideas; assess their understanding of the functional areas of business including marketing & sales, finance, human resource management, operations, and accounting, among others. Feedback from students who have participated indicates the exercise gave much more exposure and meaning to the concepts they learned in class. In this exercise, students build teams, develop a product, learn to set up a business, and design organogram, business vision, mission, and core values. The exercise empowers students to learn by doing. It accords students the opportunity to review their own knowledge and skills with respect to the concepts they have learned in the course. More than 3000 students have participated in this project since its inception in the academic year 2013/2014. It is estimated that 1000 students will participate in this project in the academic year 2017/2018.
The labor market is dynamic and frequently calls for new skills, knowledge, and abilities. The changing needs of industry place a higher demand on institutions of higher learning to monitor trends in labor needs, identify skill gaps, and to use industry insights for developing programs and curricula that mold human resources to create value for employers and society at large. While several institutions of higher learning are responsive to industry needs through curricula reviews and the development of new programs, little attention is given to pedagogical issues that affect the delivery of knowledge and the development of skills intended by various education programs. Consequently, teachers are entrusted with the freedom to decide the teaching methods that are appropriate under each circumstance. Despite the changing face of the labor market, not much energy has been channeled towards adjusting teaching methods for effective delivery of skills required by students. The failure to adjust teaching methods for training graduates has led to what is commonly known as ‘halfbaked graduates’. In other words, graduates who lack the skills and abilities necessary for placement in the industry. However, the success of an institution of higher learning is illustrated by its ability to train people who perfectly match the needs of the industry.
Culture is the constellation of shared believes, mores, values, and traditions that define the behavior of people and it is unique to each community at local and national levels. Culture determines the languages spoken by the people, their attitude towards others, and their behavior. While the family is the immediate point through which culture is learned by children, socialization at institutions such as religious organizations, places of worship, schools, and the society’s dispute resolution system reinforce culture. Unlike the Internet, traditional media in the forms of local and national print and audio-visual content tend to reinforce cultural beliefs, values, and practices of specific communities. The uniqueness of culture creates market penetration challenges to entrepreneurs in international markets. Therefore, intercultural communication is a necessary skill for reducing cultural liability and increasing the success of entrepreneurial ventures.
Multidisciplinary, multicultural, and multitasking has taken center stage in the global educational debate. Globalization and improvement in communication have affected the way organisations operate and hence influenced whom they hire. Today, it is common practice to work with people from diverse backgrounds and it requires competencies that go beyond general project management. Intercultural awareness, networking in different global communities, and learning to develop specific communication strategies for different stakeholders is all part of the package of skills and competencies that are required in today's interconnected world. This has indirect implication on the nature of skills and competencies institutions/universities must equip their students with to enable them to compete successfully in the working world.
Evaluation is of crucial importance and should meet professional standards in its design. In practice, organizational peculiarities and available resources characterize the search for the "right" approach. When used as a quality development tool, internal or self-evaluation should primarily be useful. It should generate information to answer organizational questions and provide results as a basis for discussion in decision-making processes.