Spinal Muscular Atrophy (SMA) is a neurodegenerative, inherited disease caused by an insufficient amount of functional Survival of Motor Neurone protein (SMN), though the exact mechanism underlying this is not fully understood. The primary function of SMN is assembling a ring of Sm proteins around small nuclear RNA (snRNA) in an early, cytoplasmic stage of small nuclear ribonucleoprotein (snRNP) biogenesis, a process essential in eukaryotes. SMN, together with several mRNA binding proteins, has been linked to neural transport of mRNA towards areas of growth in Motor neurons for local translation of transcripts. Previous research in our group has found that this may involve Coatomer protein-containing vesicles transported by Dynein and requiring the Sm family protein, SmB, for maintenance. Little is known, however, about what other proteins are also present and required for correct transport and localisation of these vesicles. To further investigate this, we have produced plasmids expressing each Sm protein tagged to fluorescent proteins to help track their behaviour, in some cases for the first time, and developed a detergent-free fractionation protocol to enrich for SMN containing vesicles, providing tools that can be used to further probe behaviour and interactions in the future. Using these approaches, SmN, a neural specific Sm protein, was identified to also be present in SMN-containing vesicles similarly to SmB. Analysis of the interactomes of different Sm proteins identified a novel interactor of SMN, Neurochondrin (NCDN), that appears to be required for the correct localisation of SMN in neural cells. NCDN was found to not associate with snRNPs, indicating an snRNP-independent interaction with SMN. NCDN and SMN both independently associated and co-enriched with Rab5, indicating a potential endocytic and cell polarity role for the interaction. This interaction has the potential to be key in SMA pathology and may have therapeutic potential.

本文引用Petkova(2006)所制定出來的模型及概念，探討Fama-French因子是否能作為投資機會的預測變數，並分析台灣股票市場風險溢酬(risk premium)與總體經濟變數間的關係。本文使用的總體經濟變數包括：規模效果因子(SMB)、淨值市價比效果因子(HML)、市場因子(market factor)、違約利差(default spread)、期限利差(term spread)、一個月期定存利率(RF)及股利收益率(dividend yield)。除此之外，本文亦以VAR系統表達每個狀態變數的動態特徵，討論公司治理因子與總體經濟變數(macroeconomic variables)間的關聯，並比較總體經濟變數與公司治理因子分別對股票超額報酬的解釋能力。 本研究以台灣股票市場為研究樣本，資料期間為2005年11月至2014年4月，共102個月的月報酬為研究主體。實證結果可發現：（1)本研究的價值型的股票(value stock)具有較高的風險溢酬，成長型股票(Growth stock)擁有較低的風險溢酬；而台灣上市公司也存在顯著規模效果，小市值公司具有較高的風險溢酬，大市值公司擁有較低的風險溢酬。（2)在大市值的分類下，期限利差(term spread)與淨值市價比因子(HML)呈現正向變動。在小市值的分類下，期限利差(term spread)與淨值市價比因子(HML)呈現反向變動；違約利差(default spread)則與規模效果因子(SMB)呈現正向變動。 / We use the model and concept that Petkova(2006) formulated to discuss if Fama-French factors can be the predictable variables of investment opportunity and analysis the relationship between risk premium and macroeconomic variables on Taiwan stock market. There are many macroeconomic variables in the article：Size factor(SMB)、book-to-market factor(HML)、market factor、default spread、term spread、one-month deposit interest rate and dividend yield. We capture the dynamic characters of every variables through VAR system to analyze the relationship French-French factors and macroeconomic variables. Furthermore, we compare the relationship between SMB and default spread, HML and term spread through the first step of Fama-MacBeth way. We can make two important conclusions through empirical evidence：(1) The risk premium is higher in value stocks than in growth stock, which means there is prominent book-to-market effect on Taiwan stock market. Moreover, the risk premium is higher in small firms than in large firms, which means there is magnificent size effect on Taiwan stock market.（2) The behavior that default spread has is the same as SMB factor in any book-to-market ratio criteria. However, the behavior that term spread has is the same as HML factor only in big capitalization criteria.

Simulated moving bed chromatography process (SMBCP) is the technical realisation of a countercurrent adsorption process through the cyclic port switching. SMB technology reduces the cost of packing material with high loading capacity and provides high purity and high recovery in a very short time. Major commodity applications have been found in the petroleum, food, biotechnology, pharmaceutical and fine chemical industries. The industrial applications bring an emergent demand to improve the SMBCP operation for higher product quality, productivity, efficiency and robustness. However, for this particular process, we encounter several challenges. Firstly, the interplay of the effects of strong nonlinearities, competition of solutes, mass transfer resistance and fluid dynamic dispersion produces steep concentration fronts. Mathematical model accounted for this particular property constitutes a serious difficulty for the solution procedure. Secondly, a dynamic SMB model consists of a set of partial differential, ordinary differential and algebraic equations, which are highly coupled. The large size is a problem due to its intensive computation when on-line optimisation and real-time control are necessary. Thirdly, the SMB unit operation exhibits complex dynamics. Process metrics for design and operation can be determined only when a cyclic steady state is reached after a certain number of switching. Achieving this steady state by solving the PDE models cycle after cycle involves expensive calculation. Studies have been carried out to solve these problems through process analysis, investigation on spatial discretisation techniques, and development of an accelerated integration scheme. / Through a systematic study on the advances of SMB modelling, design and control, a set of functionally equivalent models for SMBCP are identified and summarized for their practical applications. The limitations of the existing modelling techniques in industrial applications are also identified. Furthermore, structural analysis of the existing models is conducted for a better understanding of the functionality and suitability of each model. Suggestions are given on how to choose an appropriate model with sufficient accuracy while keeping the computational demand reasonably low for real time control. / Effort is made on to the systematic investigation of different numerical methods for the solution of PDEs to circumvent the steep gradients encountered in chromatographic separation. Comprehensive studies are conducted on a single column chromatographic process represented by a transport-dispersive-equilibrium linear model. Numerical solutions from the upwind-1 finite difference, wavelet-collocation, and high resolution methods are evaluated by quantitative comparisons with the analytical solution for a range of Peclet numbers. It reveals that for a PDE system with a low Peclet number, all existing numerical methods work well, but the upwind finite difference method consumes the most time for the same degree of accuracy of the numerical solution. The high resolution method provides an accurate numerical solution for a PDE system with a medium Peclet number. The wavelet collocation method is capable of catching up steep changes in the solution, and thus can be used for solving PDE models with high singularity. / The advantages and disadvantages of the wavelet based approaches are further investigated through several case studies on real SMBCP system. A glucose-fructose separation process is firstly chosen with its relatively simple isotherm representations. Simulations are conducted using both wavelet collocation and upwind finite difference methods. For more complicated applications, an enantiomers separation process is selected. As the PDEs model exhibit a certain degree of singularity, wavelet collocation and high resolution methods are adopted for spatial discretisation. It is revealed that both the wavelet based approaches and high resolution methods are good candidates in terms of computation demand and prediction accuracy on the steep front. This is the first time that these two frontier numerical methods are used for such a complex SMB system models and our results are encouraging for the development of model-based online control scheme. / In developing a new scheme to rapidly obtain the solution at steady state for any arbitrary initial condition, the concept of Quasi-Envelope (QE) is adopted under the consideration that a SMBCP can be treated as a pseudo-oscillatory process because of a large number of continuous switching. The scheme allows larger steps to be taken to predict the slow change of starting state within each switching. Combined with previously developed wavelet-based technique, this method is successfully applied to the simulation of a SMB sugar separation process. Investigations are also carried out on the location of proper starting point for the algorithm and on the effect of changing stepsize to the convergence of iteration method. It is found that if the starting state of Quasi-Envelope is chosen to be the same as the original function, the multivalue algorithm would require similar computational effort to achieve the steady state prediction, regardless of the integration stepsize. If using constant stepsize, launching QE later is helpful when quasi-envelope displays steep change at the start-up period. A changing stepsize produces slow convergence compared to the constant stepsize strategy, thus increasing the work load where the stepsize change is occurring. Other iteration method is required to be imposed to achieve faster convergence right from the beginning. Potential applications can be seen for other chemical engineering processes with inherent cyclic behaviour.

L'objectif de notre thèse est d'étudier le Business Model (BM) des PE/TPE ouest africaines à travers une approche conventionnaliste et institutionnaliste. Le BM et la convention constituent nos choix conceptuels et théoriques de départ, tandis que l'approche institutionnaliste s'est invitée via les thèmes émergents lors de l'étude. Nous avons qualifié notre conceptualisation conventionnaliste du BM de «modèle 3C» de la triple convention: convention de la valeur, convention de financement et convention d'exploitation. L'hypothèse centrale suggère que ces trois conventions lient la source de financement à la PE/TPE et que l'étude du rôle de la source de financement permettrait de comprendre le BM de la PE/TPE. L'opérationnalisation de la grille d'analyse du modèle 3C, à travers une méthodologie de recherche mixte et abductive a abouti à des résultats sur trois volets : 1) la compréhension des conventions du BM des PE/TPE nécessite un retour aux conditions préalables à celles-Ci; 2) il existe une pluralité de conventions de BM en fonction des PE/TPE, le dirigeant de la PE/TPE et des sources de financement; 3) les conventions de BM ne sont pas qu'une fonction de la source de financement et de la PE/TPE; les cadres institutionnels jouent un rôle important. Un retour sur les concepts clefs de notre recherche à la lumière de nos résultats a permis d'ouvrir des nouvelles perspectives d'étude. / The aim of our work is to study the Business Model (BM) of small and micro business (SMB) in West Africa. Our research is based on a conventionalist and institutionalist approach of the concept of BM. BM and the convention are our conceptual and theoretical initial choice, while the institutionalist approach has been introduced through the emerging topics during our research. We called our conventionalist conceptualization of BM "modèle 3C" of tree convention: value convention, financing convention and operating convention. The central hypothesis of our study suggests that these three conventions link the funding source to theSMB. So the role of funding source is relevant to study the SMB BM. The operationalization of the analytical framework of the 3C model, through a mixed methodology and abductive research has produced results in three areas: 1) understanding of the conventions of SMB BM requires a return to the preconditions; 2) there is a plurality of conventions of BM based of SMB, according to the leader of the SMB and funding sources; 3) BM conventions are not only a function of the source of funding and SMB; institutional context play an important role. A critical view of the key concepts of our research based on our findings has opened new avenues of study.

This diploma thesis deals with the analysis of launching a new IT product on the Czech market. It focuses on small businesses and introduces an IT product intended for this specific kind of a clientele. There is no such a product on the market, which leads to an excellent potential in this field. On the basis of experience, the company in which the IT product is introduced finds it crucial to develop a reliable marketing mix as well as strategy because it is difficult to offer these IT products to people owning smalls businesses who do not understand the technological aspects of such products. The theoretical part, therefore, provides the definition of the main marketing terms, B2B market, marketing strategy, marketing mix, SWOT analysis and a detailed description of launching a new product on the market, including the explanation of the differences between launching a product and service. The practical part of this paper gives information about a telephone service provider and its major competitors and it describes the IT products situation on the Czech market. The thesis also recommends a particular marketing mix of the selected product and a sale strategy. The success rate of the product is analysed on the basis of an interview with a small business and the opinion of the manager of a company in which the product is introduced.

La séparation des C8 aromatiques par Lit Mobile Simulé (LMS) permet d'obtenir du paraxylène (PX) pur. Les autres composés du mélange sont recyclés dans un réacteur afin d'être isomérisés puis séparés à nouveau. La charge du LMS est composée à environ 75% par ce flux de recyclage. L'intégration de la réaction dans le LMS, réalisée en intercalant des réacteurs d'isomérisation entre les lits d'adsorbant de la zone 3 (procédé LMS Réactif, LMSR), doit permettre une réduction de ce flux de recyclage.L'objectif de cette thèse est de développer une méthodologie d'étude des procédés de type Lit Mobile Simulé (LMS) et Lit Mobile Simulé Réactif (LMSR) basée sur :-un outil expérimental simplifié : le pilote One-column réactif (OCR)-des simulateurs One-column réactif ou non qui seront validés par les résultats expérimentaux du pilote-des simulateurs LMS et LMSR permettant d'accéder aux résultats des procédés industriels.Les simulations de One-column (OC) montrent une bonne sensibilité aux paramètres clés de la séparation des C8 aromatiques (sélectivité PX/EB et diffusion intracristalline). Les résultats expérimentaux font ressortir des difficultés importantes à mettre en œuvre le OC expérimentalement. Plusieurs hypothèses sont exposées pour expliquer les résultats obtenus mais les difficultés rencontrées limitent, en l'état, l'utilisation du pilote pour l'étude de la séparation (réactive ou non) des C8 aromatiques.L'étude du LMSR effectuée par simulation montre l'importance du nombre et de l'emplacement des réacteurs ainsi que de l'intégration du LMSR dans la boucle de production de PX. L'usage du LMSR pour la production de PX permet une réduction importante du débit de recyclage / Today, pure paraxylene (PX) is mainly obtained from a mix of C8 aromatics by a separation process based on adsorption: the Simulated Moving Bed (SMB). The other components of the blend are sent to an isomerisation reactor and are recycled to the SMB. 75% of the SMB feed flow rate come from this recycle flow. Coupling reaction and separation by inserting isomerisation reactors between the adsorption beds of the third zone (Simulated Moving Bed Reactor, SMBR) should allow a reduction of this recycling flow rate.The main objective of this thesis is to develop a new methodology for studying SMB and SMBR processes based on:- a simplify experimental tool : the One-column reactive (OCR) pilot unit- simulators of the OCR which will be validated by the experimental results obtain on the pilot unit- simulators of SMB and SMBR processes which give access to industrial processes results.Simulation results show that OC system seem to be sensitive to key parameters of C8 aromatics separation (PX/EB selectivity and micropore diffusivity). Results on the pilot unit highlight the difficulties to implement an experimental OC. Hypothesis are given to explain those results but, without modification, OCR pilot unit cannot be used to study xylene separation (with or without reaction).SMBR study done by simulation shows the impact of the placement and the number of reactors. Integration of SMBR in the global PX production scheme is also essential. SMBR allows an important reduction of recycling flow rate (up to 50%).

The fund market has grown substantially during the past decades and the majority of Swedish citizens are invested in funds directly or through pension savings. There is mixed evidence on the performance of Swedish equity funds depending on the method employed and the time period studied. In this study, we set out to estimate abnormal performance using acknowledged methods during a time-period that is both longer and more recent than previous studies. Our sample is survivorship-free and consists of 150 mutual equity funds during January 1993 to December 2011. We use a four-factor model to estimate abnormal performance compared to an index and additional risk factors. We find that the average performance is neutral net of costs and that funds outperform with 1.7 percent before costs, the difference is approximately the average management fee. Over time, we find that the average abnormal performance and the share of funds that have significant outperformance have decreased while the share of significant underperformance has increased. Since the study of fund performance started in the 1960's the twin questions has been; does funds outperform the market and is this a result of pure chance or are managers skilled? Since we observe funds with significant positive and negative abnormal performance, we want to know if the results can attributed to luck or skill. We employ the latest technique, a bootstrap simulation, to test for skill or luck. This is the first study to employ the bootstrap to distinguish skill from luck in sample of Swedish funds. By ranking funds on performance after costs, we find that the performance of the majority of funds can be attributed to skill or "bad skill". The evidence is strongest in the top 95th percentile and above, and from the bottom 50th percentile and below.

Object my master’s thesis is the design a Medical device uninterruptible power supply with managment system utilizing I2C bus. Norm EN 61000-3-2 define electric parameters for medical device power supply. In case of power supply for medial equipment is expressive accent on increased electric strength. This power supply have usually primary and secondary power circuit. Primary power circuit form line accumulator, which supplies needed output to the load and recharge reserve battery. Secondary is formed battery-pack, which serve as back-up power supply at drop-out prime circle. They have a minimum weight, small proportions, large overall effectivity also charging battery-pack and monitoring battery-pack charging condition. Result whole those master’s thesis is design Medical device uninterruptible power supply with managment system utilizing I2C bus and realization board layout. Resulting characteristics designed supply unit are: Output voltages are 5V/ 3 A , 12V/ 1,5 A and -12V/ 0,1 A. Managment support information about: Line adapter/ battery pack switch, battery-pack charging condition and actuall tempera-ture battery-pack.

This work is about setting up Samba software on server with operating system from BSD family to primary domain controller rule in the high school computer network environment with Microsoft Windows stations. After introduction to Samba software there is examined problem of Windows network based on SMB protocol. There are described steps that are necessary to setup Samba software, disk sharing services, creation of user accounts and adding client stations to domain trust. At the next there is described problematic of network printing in the Windows environment, print servers and the solution with classical printing support in FreeBSD and CUPS printing support. In the next part there is described mass installation procedure of Windows stations with same hardware configuration. At the finish, there are noticed some tasks that are necessary for next operation of installed system.

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