Smith-Magenis syndrome (SMS) is a genetic disorder caused by a deletion or mutation of the retinoic acid induced 1 (RAI1) gene on chromosome 17p11.2 that results in haploinsufficiency. SMS patients with a deletion account for 90% of the cases, while the other 10% have a mutation in RAI1. The syndrome is characterized by cognitive impairment, craniofacial abnormalities, sleep disturbances, developmental delay, obesity, and behavioral phenotypes. SMS is thought to affect 1:25,000 live births, although due to similar infantile phenotypes with Down syndrome and Prader-Willi syndrome, SMS may be mis- or under-diagnosed. In a study of 54 children, it was shown that by age 12, females with SMS are in the 90th weight percentile, while males reach the 90th percentile by age 14. It was also shown that viii teens and adults with Smith-Magenis syndrome commonly present with truncal obesity. In order to keep SMS patients healthy, to reduce the risk for future health problems associated with obesity, and to more fully understand the role of Rai1 in Smith-Magenis syndrome, it is first necessary to understand the mechanism by which SMS patients become obese. Mouse models of SMS provide a powerful tool for looking at potential mechanisms of obesity related to the haploinsufficiency of Rai1. Obesity in Smith-Magenis syndrome may result from a combination of I) behavioral, II) metabolic, and III) signaling mechanisms in which the haploinsufficiency of Rai1 causes deviations in critical pathways responsible for energy intake and expenditure. Data suggest that the Rai1+/- mice are obese and hyperphagic. Data also demonstrate that Rai1+/- mice do not have symptoms of metabolic syndrome associated with their obesity. Signaling mechanisms are deviated from normal in Rai1+/- mice, including leptin levels and the expression of Pomc, Mc4r, Bdnf, and Agrp. Treatment with ampakine drug may increase expression of Bdnf and help to control obesity in Rai1+/- mice.

Yes / This project built on research from university and employer perspectives exploring relationships between expectations of employers, professional bodies, students and universities on what constitutes a good work placement. Qualitative information on good practice was gathered in order to identify areas for improving placement practice and to illuminate employer engagement processes. Impact and benefits of different approaches to placements and curricula and/or staff Continuing Professional Development (CPD) implications were identified, together with recommendations on how improvements in understanding are shared and disseminated among employer groups and universities. Findings included that, although there were already guidelines on placement practice from a number of bodies, good practice identified at a wide range of points seemed more the result of academic staff drawing on their own experience, formed in the light of good knowledge of professional institution requirements, rather than use of guidelines. Employers mentioned professional institution requirements almost as a matter of course. That guidelines are not used as intended by those that produce them may be a general tendency, but this project¿s guidelines specifically reflect the organised voice of employers thanks to the involvement of their senior groupings, Sector Skills Councils and similar, in addition to individual employers. This project¿s guidelines are also more up to date than others identified, including particular issues affecting provision of placements that may face universities over the next few years. Students seem to recognise the benefits of placements more in retrospect than in advance. Some universities seem more flexible than others, adapting provision of placements as economic and other conditions have changed and to meet the needs of employers and placements. As one employer put it, ¿employers gravitate towards universities that make it easier¿. Employers also want to see academic credit awarded for placement learning and understandable processes with which they can help in support of this. The guidelines will be made available through a number of channels. / Royal Academy of Engineering

In January, 1951, the Lutheran Church of China denounced all relations with the American, German and Scandinavian missions, which for more than half a century had worked in the country. As one of the first, this church made a clear and corporate stand in favour of the New Democracy and the Three-Self Movement, while most of the missions made their political choice, retreated with the Nationalists and finally went to Taiwan. This book presents the Lutheran missions from optimistic new orientations in 1944 to the evacuation and the break down of the cooperation with the Chinese church seven years later. This short .period was dramatic and of great importance for the whole missionary movement, and the study may lead to renewed self-criticism and to a necessary re-evaluation of the Chinese Revolution - one of the most significant events in World History.

A city hall BRNO – North – dissertation 2015 A public building with the main function for the city hall for city part Brno – North. The building is extended with a representative hall which is also used for the culture and sociable events. Furthermore, this building includes a ceremonial hall, a cafe and a lecture room. A designed building of the city hall is situated in the centre position in a designed building. A close connection with Provaznikova street provides a connection to a current building a good accessibility for pedestrians and also for cars. At first was built the city hall, neverthless during following steps it would fit in the designed building. A position „centre“ and it´s own importance of the city hall allows to create a new strong binding with a current territory since it´s beggining. The public building and a public open space fill in this territory with an active life in a public space. A material solution of the city building reacts on a current and a future urban context. The traffic connection continues with a current communication. An entrances into the building are from Provaznikova street and a suggested square. An entrace to underground garages are on right side from a main entrance in a new designed street. A concept of the city hall includes two materials connected by communication hall which closes a public area of the square. Materials in a ground plan creates almost letter U. A combination of offices, servises for citizens, a multifunctional hall, a ceremonial hall and a cafe offer an optimal contain for a current and aslo for a future city hall. The height of building is moderate and isn´t higher than a designed or sorrounded buildings. A criterion of the building shows a kindness to people going by and also an honour to it´s meaning. A significant architectonic point of the city hall is a rotating arcade created by colonnade in an axial distance 2m and height 6,5m. This point itself contributes to an extraordinariness of this building and refers to Greek archatype. A regularity, a rast and a tectonics in all building is based on a constructive module which influenced an inner layout structure. A strenght and a stability is impared by a facade from a classical obverse masonry which has two visual solutions for an administrativelly – authoritilly part and more decorated for a part with a hall and a hall. A transparency of developments which is connected with a phylosophy of the city hall is shown in a glass entrance hall, furthermore in a solutions of corridors and offices and all kinds of boardrooms. The building is designed with a consideration of a premanently sustainable developement and management with energies, as a sustainable building.

Despite the extensive body of empirical research, the discussion on whether student employment impedes or improves educational outcomes has not been resolved. Using meta-analytic methods, we conduct a quantitative review of 861 effect estimates collected from 69 studies describing the relationship between student work experience and academic performance. After outlining the theo- retical mechanisms and methodological challenges of estimating the effect, we test whether publication bias permeates the literature concerning educational implications of student employment. We find that researchers report negative estimates more often than they should. However, this negative publication bias is not present in a subset of studies controlling for the endogeneity of student decision to take up employment. Furthermore, after correcting for the negative publication bias, we find that the student employment-education relationship is close to zero. Additionally, we examine heterogeneity of the estimates using Bayesian Model Averaging. Our analysis suggests that employment intensity and controlling for student permanent characteristics are the most important factors in explaining the heterogeneity. In particular, working long hours re- sults in systematically more negative effect estimates than not working at...

Cette thèse reconstruit la participation des jeunes et les articulations culturelles des jeunes dans les écoles ouest-allemandes à travers une collection de plus de 7 500 journaux scolaires archivés à la Bibliothek für Bildungsgeschichtliche Forschung (BBF) de Berlin. Afin d'aider à façonner la communauté scolaire et d'apprendre à agir de manière démocratique, les journaux scolaires ont été introduits après 1945 par les Alliés en collaboration avec le conseil des élèves, en particulier dans les écoles secondaires. Ce faisant, certaines préformes réussies du passé ont été poursuivies. Pour la première fois, cet ouvrage retrace l'évolution des journaux scolaires dans la première moitié du XXe siècle, puis se concentre sur les développements des années 1950 et 1960. A partir du milieu des années 1950, les rédacteurs en chef des journaux de l'école ont revendiqué avec succès leur indépendance vis-à-vis du conseil des élèves, dont les possibilités de codécision ont été désillusionnées à un stade précoce. Contrairement au conseil des élèves, les journaux scolaires ont témoigné de possibilités de co-design et d'articulation à établir nettement moins problématiques et plus fructueuses et ont ainsi contribué au développement de cultures scolaires plus fortement influencées par la libéralisation et la participation. Il est présenté dans le présent document pour les thèmes de formation au cours de la période couverte : en vue (1) de la discussion sur la technologie et les développements scientifiques et techniques pendant la Guerre froide, (2) de l'occupation avec les Etats-Unis et son influence culturelle, et (3) des confrontations avec les développements culturels des jeunes. L'ouverture croissante de l'école aux expressions culturelles des jeunes est examinée tant au niveau de la thématisation que sur le plan matériel sous la forme d'une analyse des artefacts. A cet effet, la communication symbolique sur les pages de titre des journaux scolaires sera analysée. Les efforts productifs présentés pour la liberté d'opinion dans les écoles illustrent également l'importance des journaux étudiants pour la relation enseignant-élève et le développement de structures participatives dans les écoles. Les travaux confirment la pertinence des journaux scolaires pour la reconstruction des cultures des jeunes ou des cultures des pairs dans les écoles et en tant que source productive pour les jeunes et la recherche scolaire. Les journaux scolaires sont également un phénomène international et transnational. Pour des recherches plus approfondies, ce document fournit un premier rapport de recherche complet pour l'Europe occidentale ainsi qu'une présentation pour la RDA et les Etats-Unis. / Die Arbeit rekonstruiert jugendliche Mitgestaltung und jugendkulturelle Artikulationen in westdeutschen Schulen entlang eines umfassenden Bestandes an Schülerzeitungen. Zur Mitgestaltung der Schulgemeinschaft und zum Erlernen demokratischer Handlungsweisen wurden Schülerzeitungen von den Alliierten zusammen mit der Schülermitverantwortung (SMV) insbesondere an Gymnasien eingeführt. Erstmals wird hier auch die Entwicklung der Schülerzeitungen in der ersten Hälfte des 20. Jahrhunderts nachgezeichnet. Schülerzeitungen zeugten gegenüber der Schülermitverantwortung (SMV) von deutlich unproblematischer und erfolgreicher zu etablierenden Mitgestaltungs- und Artikulationsmöglichkeiten und trugen zur Entwicklung von stärker durch Liberalisierung und Partizipation geprägte Schulkulturen bei. Dies wird für prägende Themen in der behandelten Zeit dargestellt: mit Blick auf (1) die Diskussion von Technik und naturwissenschaftlich-technischen Entwicklungen während des Kalten Krieges, (2) auf die Beschäftigung mit den USA und ihrem kulturellen Einfluss sowie (3) auf die Auseinandersetzungen mit jugendkulturellen Entwicklungen. Die zunehmende Öffnung der Schule für jugendkulturelle Ausdrucksweisen wird sowohl thematisch als auch auf materieller Ebene untersucht. Dazu wird u. a. die symbolische Kommunikation auf Titelblättern von Schülerzeitungen analysiert. Die dargestellten produktiven Bemühungen um Meinungsfreiheit in der Schule verdeutlichen auch die Bedeutung von Schülerzeitungen für das Lehrer-Schüler-Verhältnis. Die Arbeit bekräftigt die Relevanz von Schülerzeitungen für die Rekonstruktion von Jugendkulturen bzw. peer cultures im schulischen Raum sowie als ertragreiche Quelle für die Jugend- und Schulforschung. Schülerzeitungen sind darüber hinaus ein internationales und auch transnationales Phänomen. Für weitere Forschungen wird daher zudem ein erster umfassender Forschungsbericht zu Schülerzeitungen in Westeuropa, in der DDR und in den USA gegeben. / This doctoral thesis reconstructs youth participation and youth-cultural articulations at West German secondary schools. After 1945, the Allies introduced student newspapers along with student councils in order to foster the acquisition of democratic behaviors and codetermination of the school community. This project first offers a thorough documentation of the development of student newspapers in the first half of the 20th century, and then focuses on their development in the 1950s and 1960s. By the mid-1950s, the editors of the student newspapers had claimed independence vis-a-vis student councils. Student newspapers, meanwhile, bespoke far less problematic, and more successful, potentials for participation and youth-cultural articulation. They thereby contributed to school cultures more strongly influenced by liberalization and participation. The following defining themes from the era are presented in these articles: (1) the discussion of technology and natural science/technical developments during the cold war, (2) engagement with the USA and its cultural influence, and (3) involvement with youth-cultural developments. The increasing opening of schools for youth-cultural forms of expression is examined on both thematic and material levels. In addition, newspaper elements such as the symbolic communication in title pages will be analyzed. The productive efforts toward freedom of opinion in schools show the importance of school newspapers in terms of the teacher-student relationship and the development of participatory structures in schools. This project underlines the relevance of school newspapers for the reconstruction of youth cultures and peer cultures in schools in addition to being sources for youth research and school research. Moreover, school newspapers are an international and transnational phenomenon. Areas for further research are indicated in a literature review for Western Europe a consideration of the GDR and the USA.