Focal segmental glomerulosclerosis is one of the commonest cause of the nephrotic syndrome in adults patients. It is a damage of glomerulus characterized by leakage of proteins to urine and oedemas which usually develops into the end-stage renal disease within 10 years. Recently have been described familial forms of this disease which arise from injury to proteins making up filtration barrier of kidney. In 2005 non-selective ion channel TRPC6 was assigned among these proteins. In this thesis I focused on summarizing existing knowledge of the nephrotic syndrome, focal segmental glomerulosclerosis and involvement of TRPC6 in their origin. Second part of this work is devoted to the screening analysis of TRPC6 gene to discover possible mutations and polymorfisms in 47 patients with histologically proven focal segmental glomerulosclerosis or minimal change disease. The used methods were high resolution melting and direct sequencing. In the group of patients was detected no pathogenic mutation, only 2 known polymorfisms P15S and A404V and few changes which do not result in alteration of amino acid. So it seems TRPC6 gene mutations are a rare cause of the focal segmental glomerulosclerosis in adult patients in the Czech Republic.

Primary aluminum production brings about severe environmental burden due to its energy intensive process.  Secondary aluminum production contributes to cutting off high energy demand around 90-95% and greenhouse gas emission by remelting scraps. However, previous research indicates melting furnace’s energy efficiency in secondary plant is still very low, which is around 26-29% and more than 70% heat is lost in different way. The objective of this project is to investigate energy consumption and greenhouse gas (GHG) emission in secondary aluminum cast house through process analysis. The result offers a comprehensive overview to aid decision-maker to compare energy consumption and environmental impacts caused by different product or process. This project has been done in collaboration with SAPA Heat Transfer. This project consists of two tasks. First task is aimed to give an overview of annual energy distribution and carbon footprint of per ton aluminum slab in SAPA cast house. In order to analyze energy distribution, mass and energy conservation has been applied for calculation. Meanwhile, International standard method, life cycle assessment, has been used to evaluate greenhouse gas contribution of the whole production process. The second task intends to investigate two effects (melting furnace type, raw material type) on products’ energy consumption and carbon footprint.  Melting furnace’s effect is compared by selecting electric induction furnace and oxy-fuel furnace. On the other hand, raw material’s effect is studied by comparison of four different cast house products which have different raw material recipe. Calculation and analysis results indicates that per ton Sapa cast house aluminum slab consumes energy 3826MJ and contributes to 306kgCO2eq. green house gas. Meanwhile, comparison results show that oxy-fuel melting furnace has higher energy efficiency than electric induction furnace, however, it contributes much more GHG due to consumption of propane fuel. In addition, primary ingot has been concluded as distinct carbon footprint contribution than others contributors (i.e. fuel) for Sapa cast house’s slab.

This study examined the micro- and surface structures of objects manufactured by selective laser melting (SLM). The results show that the surface roughness in additively manufactured objects is strongly dependent on the geometry of the built part whereas the microstructure is largely unaffected. As additive manufacturing techniques improve, the application range increases and new parameters become the limiting factor in high performance applications. Among the most demanding applications are turbine components in the aerospace and energy industries. These components are subjected to high mechanical, thermal and chemical stresses and alloys customized to endure these environments are required, these are often called superalloys. Even though the alloys themselves meet the requirements, imperfections can arise during manufacturing that weaken the component. Pores and rough surfaces serve as initiation points to cracks and other defects and are therefore important to consider. This study used scanning electron-, optical- and focus variation microscopes to evaluate the microstructures as well as parameters of surface roughness in SLM manufactured nickel based superalloys, Inconel 939 and Hastelloy X. How the orientation of the built part affected the surface and microstructure was also examined. The results show that pores, melt pools and grains where not dependent on build geometry whereas the surface roughness was greatly affected. Both the Rz andRa values of individual measurements were almost doubled between different sides of the built samples. This means that surface roughness definitely is a factor to be considered when using SLM manufacturing.

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This thesis discusses how to test the mechanical properties of openlattice truss structures and hybrids being a tube containing a latticetruss structure. By properties we mean strength, stiffness, thermalconductivity and so forth.Mechanical testing was done on two different structures to betterunderstand how the load-bearing properties change when these structuresare subjected to tensile, compressive and bending forces. The structuresinvestigated were Diamond and Octagon built at 45° and 90°. Acousticemission was also used to evaluate and analyze the different behaviour ofthe structures. The test results were used to produce design criteria forproperties in different cell structures manufactured of Hastelloy X™. Amap with design criteria containing stiffness and weight per cubiccentimetre was produced for parts that would be subjected to compressiveforces.

During this master thesis a crushing material composite was developed in order to determine if a specific design consisting of a pointy hard phase with a ductile phase in between can reduce the load needed to crush stone and therefor decrease the energy consumption during stone crushing. The steel hard phase (ASP 2012) was printed using an additive manufacturing method called selective laser melting (SLM). A process parameter optimization was performed in order to achieve a dense material and a pre-heated building platform was used to prevent cracking. The hard phase designs were printed and then filled with bronze (JM3 and JM7). The composites and steel references were tested by placing a stone on each sample and applying pressure until the stone broke. After the tests, the loads and the deterioration of the samples was analyzed. The results showed that it was possible to print the hard phase, but some defects like micro cracking were hard to eliminate entirely. Several methods were tested to cast the bronze but the most suitable during this project was melting of the bronze in an induction furnace with vacuum atmosphere. The results from the crushing simulations showed that there was a small difference between the references and the developed macro composites. The composites began to crush stones at lower loads than the references. Nevertheless, due to a large dispersion of the results a statistical difference could not be established.

京都大学 / 0048 / 新制・課程博士 / 博士(理学) / 甲第18087号 / 理博第3965号 / 新制||理||1572(附属図書館) / 30945 / 京都大学大学院理学研究科地球惑星科学専攻 / (主査)准教授 三宅 亮, 教授 土`山 明, 准教授 河上 哲生 / 学位規則第4条第1項該当 / Doctor of Science / Kyoto University / DGAM

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