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Role of Mutations in the Essential Light Chain (ELC) of Myosin in Familial Hypertrophic Cardiomyopathy (FHC)Raytman, Alexander 09 May 2011 (has links)
Force generation and the ability of the heart muscle to contract and correspondingly to beat depends upon multiple interactions between myosin and actin-tropomyosin-troponin, the key proteins of the contractile apparatus. The myosin molecule consists of two heavy chains and two types of light chains, two essential (ELC) and two regulatory (RLC) light chains. We hypothesize that mutations in myosin ELC may affect the ability of myosin to bind to actin, thus producing structurally and/or functionally abnormal sarcomeres effecting heart muscle contraction and relaxation. We believe that this pathological process underlies the basis of Familial Hypertrophic Cardiomyopathy (FHC), a genetic disorder caused by mutations in the genes encoding the major myofilament proteins, including the myosin ELC. I have investigated the effects of two FHC ELC mutations, A57G and E143K, on the actin-myosin interaction and generation of contractile force. Here, I show evidence that mutations in the ELC may cause disruptions in sarcomeric structure which then may cause abnormal muscle contraction and lead to compensatory hypertrophy.
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Extraction and optimization for modeling ofdesalination by capacitive deionizationRehman Linder, Max, Bao, Zeshen January 2023 (has links)
Water scarcity is set to become a big challenge in the 21st century and more efficient desalinationtechnologies will be needed in the future. In this project, one desalination method called capacitivedeionization (CDI) is explored and we used a model called the ELC model to simulate CDI withComsol. The goal of this project focuses on evaluating the performance of CDI and how changingdifferent operational parameters of the process affects other aspects of desalination. Some examplesare power consumption, desalination rate and water usage. With the gathered information, the process of CDI can be optimized in some way. Even though our project simulates a specific model ofCDI, the hope is to have come to general conclusions regarding CDI so that the results can be usedfor other models. If the correlations between parameters are known, it will be easier to calibrate anysetup of CDI. The gathered data is exported, stored, processed, and plotted using Matlab functionsintegrated with Comsol. The results consist of two sets, the first for constant voltage and the secondfor constant current. Both have results on how desalination rate and energy efficiency are related toparameters such as internal voltage intervals controlling how long the desalination cycle is running,external voltage, and inflow salt concentration in the water. The key conclusions drawn are as thefollowing for constant voltage. High external voltages are effective in increasing both desalinationrate and energy efficiency but will degrade the CDI electrodes. The internal voltage span should bepretty long with high max internal voltage and the minimum internal voltage the same as the external voltage. The energy efficiency increase with lower salt concentrations in the inflow water up toa point. The best setup for the desalination rate is at quite a high maximum internal voltage withvaried low minimum internal voltage. For constant current, low current is generally efficient, whilethe maximum external voltage depends on the current. Avoid a high current with a low externalvoltage. By relating all these parameters, we get more insights into what an energy-efficient and fastadsorbing CDI setup looks like.
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Untersuchung der Pathomechanismen hypertrophieassoziierter Mutationen im MYL3 GenLossie, Janine 27 June 2012 (has links)
Myosin II, das Motorprotein des kardialen Muskels, besteht aus zwei schweren und vier leichten Ketten. Der Hebelarmbereich der schweren Myosinkette (MyHC) enthält das IQ-Konsensus-Motiv für die Bindung der essentiellen leichten Myosinkette (ELC), welche wesentlich für eine normale Kraftentwicklung des Myosinmoleküls ist. Im Rahmen dieser Arbeit wurden fünf, mit hypertropher Kardiomyopathie assoziierte, Mutationen im humanen essentiellen ventrikulären leichten Myosinketten (hVLC1)-Gen (MYL3) untersucht (E56G, A57G, E143K, M149V, R154H). Von keiner dieser Mutationen war der Pathomechanismus bekannt. Ziel der Arbeit war es, die Effekte der Mutationen im MYL3-Gen auf Proteinstruktur und Funktion zu untersuchen und daraufhin einen möglichen Pathomechanismus zu formulieren. Dazu erfolgten Strukturanalysen (CD-Spektren, Schmelzkurven, FLIM), Versuche auf Protein- und Zellebene (Protein-Protein-Interaktionsstudien, Sorting Assay) sowie Untersuchungen in vitro (Zell-Verkürzungsmessungen, isoliert perfundierte Herzen nach Langendorff) und in vivo (Echokardiographie) im transgenen Mausmodell. / Myosin II, the motor protein of cardiac muscle, is composed of two heavy chains (MyHC) and four non-covalently linked light chains (MLC). The lever arm of the MyHC contains the IQ motif that binds the essential myosin light chain (ELC), which is necessary for the normal force production of the myosin molecule. Five with HCM associated mutations in the human ventricular essential myosin light chain (hVLC1) -gen (MYL3) were investigated in this study (E56G, A57G, E143K, M149V, R154H). The pathomechanisms of the mutations were not known. Aim of the study was i) to test the hypothesis that mutations in the ventricular essential myosin light chain affect the protein structure, the binding to the IQ motif of MyHC and the force production of the myosin molecule as well as ii) to postulate an accompanying pathomechanism. Structural analyses (circular dichroism, melting curves, fluorescence lifetime imaging microscopy), functional investigations (surface plasmon resonance spectroscopy, sorting assay) and in vivo (echocardiography) and in vitro studies in a transgenic mouse model were performed.
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Neuaufbau eines Pelletron-Beschleunigers und Untersuchungen zum Laserhydrieren von Silizium / Rebuild of a pelletron accelerator and investigations on laser hydriding of siliconSchwickert, Marcus 29 October 2002 (has links)
No description available.
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Developing A Teachers' Handbook for Content-based Instruction at Brigham Young University's English Language CenterHardman, Melinda Clement 22 April 2009 (has links)
In Winter semester of 2005 the English Language Center (ELC) began plans to implement a content-based program for students at its highest proficiency level in order to provide them with more effective preparation for academic studies in English. Since that time, the program has undergone various changes to provide a more stable, cohesive, and responsive curriculum. There is a need, however, for greater teacher support and training. This paper outlines the process I underwent to create a handbook that would provide needed guidance and training for teachers in this program. This project involved an in-depth needs analysis of teachers and students in the program. Results from this analysis led to the selection of three basic principles that were used in the design and development of this project: (1) practicality, (2) instructiveness, and (3) sustainability. The resulting project is a binder organized into four tabs: (1) Program Overview, (2) The Basics of Content-based Instruction at the ELC, (3) Challenges and Strategies in CBI, and (4) Additional Resources. This resource was developed using Microsoft Word 2007 and includes a variety of graphics, text boxes and layouts to provide a professional and user-friendly product. An evaluation of the project based on the responses of three teachers who used the handbook during Winter semester 2009 revealed that it was a useful and needed resource for teaching content-based classes at the ELC. Further piloting is needed to verify these findings. It is recommended that in the future this resource be maintained by the ELC Coordinator assigned to oversee the content courses. As the ELC continues to refine the content curriculum, evaluation and revisions of this handbook should be carried out at regular intervals to ensure its continued relevance and accuracy over time.
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