This master thesis deals with the teaching of pronunciation in primary schools in the Czech Republic. The theoretical thesis explains the importance of pronunciation, of phonetics and of phonology, because of achieving communication skills. It explains terms, that are related with pronunciation, describes phenomena in the German phonetic system, that are difficult for Czechs, and discusses methods of teaching German pronunciation in the context of primary school. It also points out that the inclusion of teaching German pronunciation is insufficient in RVP and very diverse in the school educational plans. The practical part of the thesis introduces three textbook files and analyses the degree of the inclusion of pronunciation in their concepts. Afterwards, it shows research that took place in five classes in primary school. It conducts observations and interviews with teachers in this research. The mastery of the very difficult German sounds by the pupils was found out. Finally, the results of the research in all classes are compared. The results of the research show that there is some connection between the teacher's teaching skills and the pupil's pronunciation skills. However, pupils don't manage the pronunciation of German sounds after two years of learning German. KEYWORDS German as a foreign...

This project describes design of the system for diagnosis Parkinson’s disease based on speech. Parkinson’s disease is a neurodegenerative disorder of the central nervous system. One of the symptoms of this disease is disability of motor aspects of speech, called hypokinetic dysarthria. Design of the system in this work is based on the best known segmental features such as coefficients LPC, PLP, MFCC, LPCC but also less known such as CMS, ACW and MSC. From speech records of patients affected by Parkinson’s disease and also healthy controls are calculated these coefficients, further is performed a selection process and subsequent classification. The best result, which was obtained in this project reached classification accuracy 77,19%, sensitivity 74,69% and specificity 78,95%.

The subject of this diploma thesis is engineering design of the sediment conveyer. Conveyer is instrumental to total emptying of sediment silo that is situated in a sewage treatment plant. Proposed construction has to fulfill the specified functional requirements and parameters. Parts of solution are also necessary analytical and FEM calculations.

The dissertation thesis deals with signal detection by an ionization detector in the environmental scanning electron microscope and utilization of this detector to gain required information in a specimen image. Main interest is focused on the detector containing several electrodes with a varied geometry arrangement and voltages on these electrodes. The detector was named segmental ionization detector. Detection capabilities of the segmental ionization detector were studied through computer simulations and experiments in the microscope utilizing knowledge from a technical literature background. On the base of the accomplished experiments, the segmental ionization detector has been optimized for the secondary electron detection improvement and at another configuration optimized for a high material contrast acquisition of the specimen image. Consideration of benefits of the examined segmental ionization detectors is included in the work.

Genome composition and architecture is shaped by two types of processes: those that introduce heritable changes (mutagenesis) and those that determine the fate of such changes in the populations (genetic drift and selection). Chemical and biological properties of mutagenesis determines the frequencies at which different type of mutations occur, which, in turn, determines their rates of fixation by drift and affects the spectrum of mutations available for selection to operate on. As the result, genomes of living organisms carry many signatures mutagenesis.

Nephrotic syndrome is characterized by proteinuria, hypoproteinemia, edemas and hyperlipidemia. It occurs in primary (e.g. focal segmental glomerulosclerosis, FSGS or minimal change disease, MCD) and in secondary glomerulopathies (e.g. kidney amyloidosis). In primary forms, great attention is paid to the potential genetic background of the disease and due to new molecular genetic methods genes, whose mutations cause different nephropathies (e.g. ACTN4 or INF2) were identified. The aims of presented doctoral thesis were following. Firstly, to continue the mutational analysis of ACTN4 that was described in the author's diploma thesis in other glomerulopathies. Secondly, to implement the mutational analysis of INF2 and subsequently analyse this gene in patients with FSGS/MCD and in patients from special group characterized by positive family history for end stage renal disease (ESRD) in combination with advanced chronic kidney disease (CKD) or already developed ESRD at the time of diagnosis. Thirdly, mutational analysis of NPHS2 and TRPC6 (methods implemented in laboratory earlier) in selected patients from the special group. Finally, expression analyses of genes important for podocyte function or connected with human immune system. This part also verifies the applicability of NPHS2/SYNPO expression...

Objectif: Nous avons effectué une étude chez 135 patients ayant subis une chirurgie lombo-sacrée avec vissage pédiculaire sous navigation par tomographie axiale. Nous avons évalué la précision des vis pédiculaires et les résultats cliniques. Méthodes: Cette étude comporte 44 hommes et 91 femmes (âge moyen=61, intervalle 24-90 ans). Les diamètres, longueurs et trajectoires des 836 vis ont été planifiés en préopératoire avec un système de navigation (SNN, Surgical Navigation Network, Mississauga). Les patients ont subi une fusion lombaire (55), lombo-sacrée (73) et thoraco-lombo-sacrée (7). La perforation pédiculaire, la longueur des vis et les spondylolisthesis sont évalués par tomographies axiales postopératoires. Le niveau de douleur est mesuré par autoévaluations, échelles visuelles analogues et questionnaires (Oswestry et SF-36). La fusion osseuse a été évaluée par l’examen des radiographies postopératoires. Résultats: Une perforation des pédicules est présente pour 49/836 (5.9%) des vis (2.4% latéral, 1.7% inférieur, 1.1% supérieur, 0.7% médial). Les erreurs ont été mineures (0.1-2mm, 46/49) ou intermédiaires (2.1 - 4mm, 3/49 en latéral). Il y a aucune erreur majeure (≥ 4.1mm). Certaines vis ont été jugées trop longues (66/836, 8%). Le temps moyen pour insérer une vis en navigation a été de 19.1 minutes de l΄application au retrait du cadre de référence. Un an postopératoire on note une amélioration de la douleur des jambes et lombaire de 72% et 48% en moyenne respectivement. L’amélioration reste stable après 2 ans. La dégénérescence radiologique au dessus et sous la fusion a été retrouvée chez 44 patients (33%) and 3 patients respectivement (2%). Elle est survenue en moyenne 22.2 ± 2.6 mois après la chirurgie. Les fusions se terminant à L2 ont été associées à plus de dégénération (14/25, 56%). Conclusion: La navigation spinale basée sur des images tomographiques préopératoires est une technique sécuritaire et précise. Elle donne de bons résultats à court terme justifiant l’investissement de temps chirurgical. La dégénérescence segmentaire peut avoir un impact négatif sur les résultats radiologique et cliniques. / Objective: The authors studied 135 consecutive patients following a lumbo-sacral fixation using pedicle screws and CT-based navigation to evaluate pedicle screw accuracy and clinical outcomes. Methods: The series included 44 men and 91 women (mean age 61 years, range 24-90 years). All 836 screws were planned with pre-operative CT-Scans in a navigation system (SNN, Surgical Navigation Network, Mississauga, Ontario, Canada) for diameter, length and direction. Fixation included the lumbar spines only (55), the lumbo-sacral spine (73) or the thoraco-lumbo-sacral spine (7). Pedicle perforation, screw length and spondylolisthesis were assessed on post-operative CT-Scan. Pain was surveyed using self-rated scales, visual analogue scales, Oswestry and SF-36 questionnaires. Bony union was assessed on post-operative follow-up radiographs. Results: Pedicle perforation was found in 49/836 (5.9%) screws (2.4% laterally, 1.7% inferiorly, 1.1% superiorly, 0.7% medially). The errors were minor (0.1-2mm, 46/49) or intermediate (2.1 – 4 mm, 3/49). All intermediate errors were lateral. There were no major errors (≥ 4.1mm). Some screws were judged too long (66/836, 8%). The average time to insert one screw with navigation was 19.1 minutes from application to removal of the reference frame. The amount of improvement at one year post-operation for self-rated leg and back pain were 72% and 48% respectively. The improvement was stable over 2 years. Above-level and below-level radiological degenerations were found in 44 patients (33%) and 3 patients respectively (2%) and occurred on average 22.2 ± 2.6 months after the surgery. Fusions ending at L2 had the most degenerations (14/25, 56%). Conclusion: CT-based preoperative navigation for lumbo-sacral pedicle screw insertion is accurate and associated with a good short term outcome, making it worth the investment of the additional time required. Segmental degeneration may have a negative effect on radiological and clinical outcomes.

本研究旨在調查電腦輔助英語發音學習軟體 「MyET」，對學習者在學習英語發音方面的影響。 利用電腦輔助英語發音學習軟體(CAPT)，練習英語的類化效果，也列為調查重點之一。 此外，學生使用CAPT過程中遭遇的困難和挑戰，以及互動過程中發展出來的對策也一一加以探討。 本研究的目的是要把CAPT在英語聲韻教學的領域中做正確的定位，並且探討如何使用其他的中介工具(例如人類)來強化此類軟體的輔助學習效果。 參與本次研究的大學生一共有九十名，分為三組：兩組CAPT組(亦即實驗組，使用CAPT獨自或與同儕一起使用CAPT學習英語發音)、非CAPT組(控制組)一 組。每組三十名。實驗開始，所有學生以十週的時間練習朗讀 從「灰姑娘」(Cinderella) 摘錄的文字，此段文字由發行 MyET 的公司線上免費提供。 實驗前與實驗後，兩組的學生各接受一次測驗。 每週練習結束後，學生必須將學習心得記載於學習日誌上；教師也針對每個學生的學習心得給予指導回饋。 研究結果顯示，兩個CAPT組別(亦即使用CAPT發音學習軟體的組別)的學生在學習英語聲韻的過程中，都有明顯及正面的進步與改變。尤其是語調與速度快慢方面的進步遠勝於發音的進步。再者，實驗組學生以十週的時間利用CAPT學習英語後，在朗讀新的文字時，無論是發音或語調都有類化的效應，但是在速度快慢方面則無顯著進步。然而，實驗結果三組的發音表現，在量化統計上並未達到明顯的差異。 雖然如此，在質化的探究上，經過分析學生的學習心得後得知：所有組別當中，獨自使用CAPT學習英語發音的組別，最能夠自我審視語言學習歷程 (包括模仿和學習樂趣)。至於共同使用CAPT學習的學生自述在英語流暢度、語調及發音方面獲致最大的改善。控制組的學生因為沒有同儕的鷹架教學及回饋，也沒有 MyET提供的練習回饋，練習過程中，學生自述學習困難的頻率最高，學生也認為學習收穫很少。 參與本次研究實驗組的學生認為， CAPT提供練習回饋的機制設計有改進的空間。 有關本研究結果在理論及英語教學上的意涵以及研究限制，於結論當中一一提出加以討論。 關鍵字：電腦輔助語言教學，語音辨識軟體，超音段，語調，時長，學習策略， 中介 / This present study investigated the impact of computer-assisted pronunciation training (CAPT) software, i.e., MyET, on students’ learning of English pronunciation. The investigation foci included the generalization of the effect of practice with the CAPT system. Also examined are the difficulties and challenges reported by the students who employed the CAPT system and the strategy scheme they developed from their interaction with the system. This study aimed to position the role of the CAPT system in the arena of instruction on English pronunciation and to investigate how other kinds of mediation, such as that of peer support, could reinforce its efficacy. This study involved 90 Taiwanese college students, divided into two experimental groups and one control group. The two experimental groups practiced English pronunciation by using a computer-assisted pronunciation training (CAPT) program either independently or with peers while the control group only had access to MP3 files in their practice. All the groups practiced for ten weeks texts adopted from a play, Cinderella, provided by MyET free of charge on line. They all received a pretest and a posttest on the texts they had practiced and a novel text. Each week after their practice with the texts, the participants were asked to write down in their learning logs their reflections on the learning process in Chinese. In the same way, the instructor would provide her feedback on the students’ reflections in the logs every week. The results showed that the ten-week practice with the CAPT system resulted in significant and positive changes in the learning of English pronunciation of CAPT groups (i.e., the Self-Access CAPT Group and the Collaborative CAPT Group). The progress of the participants in intonation and timing was always higher than in segmental pronunciation. Moreover, the ten-week practice with the CAPT system was found to be generalized (though the generalization is less than mediocre) to the participants’ performance in the production of segmental pronunciation and intonation but not in the timing component in reading the novel text. However, the improvement of the CAPT groups was not great enough to differentiate themselves from the MP3 Group. Though the quantitative investigation did not reveal significant group differences, the qualitative analysis of the students’ reflections showed that the learning processes all the three groups went through differed. The Self-Access CAPT Group outperformed the other two groups in developing self-monitoring of language learning and production, and in enjoying working with the CAPT system/texts. Among the three groups, the Collaborative CAPT Group outscored the other two groups in reporting their gains and improvement in fluency, intonation and segmental pronunciation, as well as developing strategies to deal with their learning difficulty. Though the students in the MP3 group also made significant progress after the practice, without peers’ scaffolding and the feedback provided by MyET, they reported the highest frequency of difficulties and the least frequency of gains and strategies during the practice. The participants of this study also considered necessary the improvement of the CAPT system’s feedback design. At the end of the study theoretical and pedagogical implications as well as research limitations are presented. Key words: Computer-Assisted Language Learning (CALL), Automatic Speech Recognition System (ASRS), segmental pronunciation, prosody, intonation, timing, learning strategies, mediation

En aquest treball es presenta l'evolució molecular i estudi funcional de gens localitzats a les duplicacions segmentàries de la regió 7q11.23, implicada en la Síndrome de Williams-Beuren (SWB). S'ha datat l'aparició d'aquestes duplicacions en els últims 25 milions d'anys d'evolució i s'ha proposat un model evolutiu amb reordenaments específics i mecanismes de generació. Correlacions clínico-moleculars en els pacients amb la SWB han permès determinar que l'haploinsuficiència per NCF1, un gen localitzat a les duplicacions, és un factor protector per hipertensió. S'ha proposat un model patogènic per la hipertensió, implicant l'oxidasa NAD(P)H i estrès oxidatiu, suggerint que noves estratègies terapèutiques podrien ser utilitzades. A més, s'ha caracteritzat parcialment la funció de GTF2IRD2, un altre gen de les duplicacions. GTF2IRD2 interacciona amb altres factors de transcripció relacionats, té una localització subcel·lular variable i no s'uneix a ADN. Aquests resultats contribueixen a conèixer millor els mecanismes mutacionals i patogènics de la SWB. / This work presents the molecular evolution along with the functional analysis of the genes located in the segmental duplications flanking the 7q11.23 region, involved in Williams-Beuren syndrome (WBS). The generation of the segmental duplications has been dated to the last 25 million years of evolution and an evolutionary model with specific rearrangements and mechanisms has been proposed. Clinical-molecular correlations in WBS patients have allowed to determine that haploinsufficiency at NCF1, a gene located in the duplications, is a protective factor for hypertension. A pathogenic model for hypertension has been proposed, implicating NAD(P)H oxidase and oxidative stress, and suggesting that novel therapeutic strategies could be used. In addition, the functional characterization of another gene of the duplications, GTF2IRD2, has been partially achieved. GTF2IRD2 has been shown to interact with other related transcription factors, to display variable subcellular localization and to lack DNA binding properties. These results contribute to a better knowledge of the mutational and pathogenic mechanisms of the WBS.

In der vorliegenden Arbeit wurde die Ringfuge einer Tübbingschale hinsichtlich der Möglichkeit einer Reibungskopplung zur Übertragung von Scherkräften, wie sie bei relativen Ringversätzen auftreten können, untersucht. Ein Schwerpunkt lag dabei in der Ermittlung von Scherwiderständen an unterschiedlich profilierten Betonprobekörpern mit direktem Betonkontakt bzw. mit Verwendung einer Zwischenlage. Neben diesen Scherversuchen wurde zudem die Entwicklung der Normalkraft in der Ringfuge untersucht. Dabei wurden zur Verfügung gestellte Messdaten eines In-situ-Versuchs am Neuen Schlüchterner Tunnel ausgewertet. Weitere Erkenntnisse lieferte ein unter Laborbedingungen durchgeführter Bauteilversuch an Originaltübbings. Letztere wurden mittels Spanngliedern über einen Zeitraum von drei Jahren verspannt. Der Spannkraftverlust infolge von Kriechen und Schwinden des Betons sowie der Relaxation der Dichtungsprofile und der Spannglieder wurde kontinuierlich ermittelt