Skipping of exon 30 in C5 gene results in complete human C5 deficiency and demonstrates the importance of C5d and CUB domains for stability

The deficiency of complement C5 is rare and frequently associated with severe and recurrent infections, especially caused by Neisseria spp. We observed the absence of component C5 in the serum of 3 siblings from a Brazilian family with history of consanguinity. The patients had suffered from recurrent episodes of meningitis and other less severe infections. Sera from these patients were unable to mediate hemolytic activity either by the classical or alternative pathways and presented extremely low levels of C5 protein (13, 0.9 and 1.0 mu g/ml-normal range: 45-190 mu g/ml). Hemolytic activity could be restored by the addition of purified C5 to deficient serum. Sequencing of sibling C5 cDNA revealed a homozygous 153 bp deletion that corresponds precisely to exon 30. The parents carried the same deletion but only in one allele. Sequencing of the corresponding region in the genomic DNA revealed a C to C substitution within intron 30 and, most significantly, the substitution of GAG(4028) for GAA(4028) at the 3` end of exon 30 which is most likely responsible for skipping of exon 30. The resulting in-frame deletion in the C5 mRNA codes for a mutant C5 protein lacking residues 1289-1339. These residues map to the CUB and C5d domains of the C5 alpha chain. This deletion is expected to produce a non-functional and unstable C5 protein which is more susceptible to degradation. (C) 2009 Published by Elsevier Ltd.

Complete assignment of (1)H and (13)C NMR spectra of alpha-phenylsulfinyl-N-methoxy-N-methylpropionamide and some p-substituted derivatives

The complete assignment of the (1)H and (13)C NMR spectra of the diastereomeric pairs of some alpha-arylsulfinyl-substituted N-methoxy-N-methylpropionamides with the substituents methoxy, methyl, chloro, nitro is reported. Copyright (C) 2008 John Wiley & Sons, Ltd.

Performance Evaluation of Different PV-Array Configurations under Weak Light Conditions and Partial Shadings

The paper analyses empirical performance data of five commercial PV-plants in Germany. The purpose was on one side to investigate the weak light performance of the different PV-modules used. On the other hand it was to quantify and compare the shading losses of different PV-array configurations. The importance of this study relies on the fact that even if the behavior under weak light conditions or the shading losses might seem to be a relatively small percentage of the total yearly output; in projects where a performance guarantee is given, these variation can make the difference between meeting or not the conditions.When analyzing the data, a high dispersion was found. To reduce the optical losses and spectral effects, a series of data filters were applied based on the angle of incidence and absolute Air Mass. To compensate for the temperature effects and translate the values to STC (25°C), five different methods were assessed. At the end, the Procedure 2 of IEC 60891 was selected due to its relative simplicity, usage of mostly standard parameters found in datasheets, good accuracy even with missing values, and its potential to improve the results when the complete set of inputs is available.After analyzing the data, the weak light performance of the modules did not show a clear superiority of a certain technology or technology group over the others. Moreover, the uncertainties in the measurements restrictive the conclusiveness of the results.In the partial shading analysis, the landscape mounting of mc-Si PV-modules in free-field showed a significantly better performance than the portrait one. The cross-table string using CIGS modules did not proved the benefits expected and performed actually poorer than a regular one-string-per-table layout. Parallel substrings with CdTe showed a proper functioning and relatively low losses. Among the two product generations of CdTe analyzed, none showed a significantly better performance under partial shadings.

Narration in Swedish pre- and primary school : a recourse for language development and multilingualism

Increased immigration in Europe and worldwide has led to more pre- and primary school students being educated through the medium of a second language, and there is considerable research, much of it coming from Australia, to suggest that in order to cope with this situation, children will need to begin to acquire, from their earliest years in pre-school, a variety of knowledge-based language skills that will be sufficient to carry them through the subject-based education they will encounter in their subsequent schooling. This is particularly important for L2-students who are less likely to meet academic language outside the school. In this paper, based on transcripts of oral interactions in the classroom, it is argued that conversational and story-telling skills, oral and written, provide a rich environment for the development of academic school language, while at the same time promoting and making good use of the cultural diversity that is increasingly a feature of pre-primary and primary classrooms.

Simulation study of cascade heat pump for solar combisystems

This paper focuses on the study of cascade heat pump systems in combination with solar thermal for the production of hot water and space heating in single family houses with relatively high heating demand. The system concept was developed by Ratiotherm GmbH and simulated with TRNSYS 17. The basic cascade system uses the heat pump and solar collectors in parallel operation while a further development is the inclusion of an intermediate store that enables the possibility of serial/parallel operation and the use of low temperature solar heat. Parametric studies in terms of compressor size, refrigerant pair and size of intermediate heat exchanger were carried out for the optimization of the basic system. The system configurations were simulated for the complete year and compared to a reference of a solar thermal system combined with an air source heat pump. The results show ~13% savings in electricity use for all three cascade systems compared to the reference. However, the complexity of the systems is different and thus higher capital costs are expected.

Distance teacher education – some experiences from Dalarna University, SwedenPaper presented at the 33rd Annual ATEE Conference Brussels, Belgium, 23rd – 27th August 2008

Distance teaching is now-a-days used in different shapes. However, it is something different from traditional campus organised education as it systematically uses Information Communication Technology (ICT) as a key element. When the distance teacher education started in Sweden many teacher educators doubted the wisdom of this. They expressed that the educational process to become a teacher would be deteriorated. For instance, they feared for high drop out rates and difficulties to examine in a proper way. The Swedish National Agency for Higher Education has recently edited a report that showed that this form of teacher education was well adapted to the labour market, but the possibilities for the teacher students to shape their education were relatively limited. However, we still know quite little about the effects of this way to educate teacher students. This paper explores the possibility of using distance teacher education.In a case study 20 students, who were the first to finish a complete a distance teacher education at Högskolan Dalarna, were asked in a questionnaire how they had apprehended their education. We also interviewed four of these students, as well as five teacher educators.One of our findings were that the distance teacher education reached new target groups, who not had been able to participate in university studies if it not had been offered in this form. Especially, this was valid for the middle-aged women, living a long distance away from a university, with social responsibilities for children or old parents. Other findings were that these students in general were target oriented and ambitious, wrote more than the campus students and developed that kind of skill better. Marratech, an ICT system for small groups, e-mail and chat were used for the communication. Marratech was considered to permit free and spontaneous communication, both of the teacher educators and the students. Initially the teacher educators were sceptical to distance teacher education, but afterwards they were surprised of how well it had worked. They declared that they had better and nearer contact with their students and more control over the students´ performance, but some parts of the teacher education were better suitable for campus education, for example, power of creating characters. Distance teacher education was considered time consuming and demanded much activity from the teacher educators as the students wanted rapid responses. This study indicates that distance teacher education works well for mature individuals with high motivation. However, it demands more time from the teacher educators, but it gives in general good results. Still, there are pedagogical challenges to overcome. Maybe we should reflect on a mix of distance teacher education and campus based teacher education, instead of separated ways of accomplishing teacher education?

Modeling Of District Metered Areas With Relatively High Leakage Rate. The Case Study Of Kalipoli’s DMA

The article reviews the modelling of District Metered Areas (DMAs) with relatively high leakage rate. As a generally recognised approach in modelling of leakage does not exist, modelling of leakage by enginners and other researchers usually takes place by dividing the whole leakage rate evenly to all available nodes of the model. In this article, a new methodology is proposed to determine the nodal leakage by using a hydraulic model. The proposed methodology takes into consideration the IWA water balance methodology, the Minimum Night Flow (MNF) analysis, the number of connections related to each node and the marerial of pipes. In addition, the model is illustrated by a real case study, as it was applied in Kalipoli’s DMA. Results show that the proposed model gives reliable results.

The human side of project management - an investigation of critical chain concepts in the Argentinean Project Environment

The global marketplace is rapidly intensifying. Longer product sales lives, greater profit margins or simply survival, is dependent on management¿s ability to create and lead change. Project Management has become an important competency, combined with other business practices to adapt to the trend of changing conditions. Critical Chain is a relatively new project methodology, elaborated by Eliyahu Goldratt in order to complete projects faster, make more efficient use of resources and securing the project deliverables. The methodology is based on the assumption that traditional project techniques such as CPM and PERT, do not recognize critical human behavior. The methodology claims that many project failures are a direct result of how safety is built into the task delivery times, and then wasted by human behavior such as Student Syndrome, Parkinson Law and Multitasking. However, there has been little or no previous research regarding this topic in the Argentine marketplace. This study intended to investigate to what extent the human behavior concepts of critical chain project management are present, by performing in-depth interviews with Argentine project stakeholders. It appears that the four human behavior concepts are present in Argentina and that the majority of Argentine companies are yet to apply project management techniques.

Measures of the welfare cost of inflation: a complete ordering

This paper presents three contributions to the literature on the welfare cost of ináation. First, it introduces a new sensible way of measuring this cost - that of a compensating variation in consumption or income, instead of the equivalent variation notion that has been extensively used in empirical and theoretical research during the past fiftt years. We Önd this new measure to be interestingly related to the proxy measure of the shopping-time welfare cost of ináation introduced by Simonsen and Cysne (2001). Secondly, it discusses for which money-demand functions this and the shopping-time measure can be evaluated in an economically meaningful way. And, last but not least, it completely orders a comprehensive set of measures of the welfare cost of ináation for these money-demand specification. All of our results are extended to an economy in which there are many types of monies present, and are illustrated with the log-log money-demand specification.