Inherited metabolic disorders (IMD) form a diverse group of several hundred different diseases with a relatively high cumulative incidence (stated up to 1:600). They are associated with accumulation of the substrates and lack of the products in specific metabolic pathways, which is caused by deficiency of the enzyme or its activator, or dysfunction of the transport protein. However, the underlying cause is at the DNA level. The grounds for different phenotype manifestation in patients with the same genotype are often not known. During my work at the Institute of Inherited Metabolic Disorders, I designed several new methods for the research of IMD and applied them in the patients and their families. I created procedures for the isolation of lysosomal membranes that are used for the research of lysosomal storage disorders and general properties of lysosomes. Next, I introduced several novel assays for determination of the X-inactivation ratio, which led to a significant increase of informative women. Nowadays, we use these methods in heterozygous women with X-linked diseases in order to study the influence of X-inactivation on the manifestation of the diseases. The cases of a girl with mucopolysaccharidosis type II, a girl with OTC deficiency and a family with the mutation in HPRT1 gene are described...

This thesis is covering the Convention of the rights of the child, its consequences and use in pedagogical theory and practice according to the paragraph 42 of this convention. It has three main parts, which are aiming for following the logical consequence of the topic compilation. First part is covering theoretical base of the Convention, starting with its historical development and current state, legal position, institutional arrangement, approaches to the Convention by selected European countries, including analysis of individual articles and it also presents commentary to these articles in connection with legal regulations in Czech Republic and finding the causes and deficiencies of its implementation, observing to it and its fulfilling. Second part is covering the definition of article 42 of the Convention as to the further education of adults as a tool for exercising the rights of children and to other theoretical and practical possibilities of its further efficient dissemination. Third part is related to that as it provides practical exercises, directions and games, how to get children acquainted with the document of the Convention not only during the school, but also within their free-time activities, including the chance for children to take part in the children{\crq}s parliaments or establishing own school self-governments and thus participate on public matters.

The thesis targets at events which took place in the town of Prachatice and its surroundings at the end of the Second World War. It is based on written sources (filed in the State District Archives Prachatice), as well as on literature sources focusing on this topic and eyewitness memories. The first part presents a brief characterization of the town from geographical, social and economic point of view. The second chapter deals with events taking place in September 1938 when Prachatice was occupied by Nazi Germans. The third part shortly depicts the process of front war situations in Europe. The main part of the thesis consists of chapter 4 and 5 which present the way of living and events happening in the town from January 1945 to May 1945 when Prachatice was liberated by the US army. The thesis aims to connect general relations with events that happened in Prachatice and its surroundings not only to illustrate the topic from literature sources but also from people{\crq}s own memories. It tends to depict this period full of worries and hope. It is hoped to contribute to completion of the town history.

The thesis Radio Broadcasting for the German Minority in the Czech Countries until the Year 1945, with the Example of the Melnik Broadcast Transmitter describes the radio broadcasting for the German people living in Bohemia and Moravia in the period from its formation until the year 1945. It concentrates on the radio broadcaster in Melnik that was built in 1938 with the purpose of giving a special broadcasting to the German minority living in the Czech countries. The analytical part is divided into three sections: broadcasting for the Germans in the First and Second Republic and Protectorate of Bohemia and Moravia. Another chapter focuses on the broadcasting from The Soviet Union (especially on the broadcaster Sudetendeutscher Freiheitssender) and Great Britain. It focuses on the contrasting access to the radio broadcasting for this group from the side of the Czech political representation and from the side of the German state. In the Czechoslovakia, there was this broadcasting in hands of the private institution Urania. The Sudeten Germans have focused their attention to the German stations, which were offering them more popular program. This study describes the influence of the German propaganda on the Sudeten Germans behaviour and the reaction of the Czechoslovakian Broadcasting Company. It...

The doctoral dissertation thesis is focused on assessment of information systems and defines the main deficiencies in operation and management of information systems in relation to processes in the department of informatics with partial overlapping into the other parts of the company. The thesis further extends the current view of assessment of information systems in the company and, in accordance with the goal of the work, it defines its own methodology, which defines the specific assessment process and subdivides its contents into individual units. The proposed methodology provides an overview of the deficiencies and possible measures in operation and management of information systems. Qualitative verification of methodology in small, medium and large companies using case studies form a part of the thesis as well.

Information system, methodology B2EPUS, questionnaire, analysis HOS8, analysis SWOT

The effective surveillance of large critical areas is crucial for their security and privacy. There is no publicly available and acceptable solution of automating this task. This thesis aims to create an application utilizing a combination of a pan-tilt robotic manipulator and a visible-spectrum camera. Based on the pan-tilt unit's position and camera's images, the application searches for semantically significant changes in the captured environment and marks these regions of interest.

The thesis theme is the design of 3 MW high-speed permanent magnet synchronous motor. The work is divided into several parts, and contains the information given on the topic. The first part lists trends and new technologies of high-speed electric machines, and the issues and construction of high-speed synchronous machines are also listed here. In the second part, the machine is analytically designed from the specified values, which is then modelled in the RMxprt program. In the penultimate part, the engine is simulated by programs that are included in the ANSYS Electronics Desktop package, they are RMxprt and Maxwell. The modified engine model in RMxprt is simulated using defined formulas and then converted to Maxwell 2D, where the engine is simulated using the finite element method. In the last part, the results from the analytical calculation are compared with other high-speed machines. In addition, the results of simulations from RMxprt and Maxwell were compared in the last part.

The theme of Master Thesis is the Brno dam and its effective utilization. The very subject of this thesis is the Cultural and Social Center (the Center). The project follows a broad urban context and provides a new insight into the operation and use of this site. It contains a proposal of movement coordination and effective utilization of the most attractive areas closely related to the water surface. Cultural and Social Center is designed as a part of the landscape. It connects two levels of trails leading around the object. This connection is expressed through the dominant stairs. The program of the Center complements and evaluates leisure activities of the site, plus the Center offers an extraordinary space. Digital RGB spectrum system senses the current weather and converts the results into a graphic and scenic projection. Thanks to this system a new concept of space is continuously formed. The atmosphere, temperature, texture, intensity etc.. The visitor thus will never be a part of the same "exhibition." The first floor, (level +0,00m) around the water surface, is preferably used as a Cultural and Social Center. It has three naves of the Center, foyer with cafe and snacks, as well as it covers a DPMB building of cabeling. Few parts of the facade are designed as a sliding facade, thus visitors could stay in direct contact with the water surface and landscape. Second floor – (level +3,50m) is an spatial continuation on the Center and the rest is mostly used for technical security of the building. In foyer there is a second part of cafe – more private and relaxing room. Third floor (level +7,00m) is connected with the trail going around the building. At this level takes place leisure and educational center, which is complemented by the multifunction hall and an administrative facilities. The idea of landscape use is to provide an outdoor sports urban park a la Miralles urban parks.

The aim of this diploma thesis is to analyse development in arranging of the real estate transactions in the Czech Republic after 1989 and describe contemporary condition. Contemporary condition was analysed as unsatisfactory. There does not exist any law that would regulate real estate market, neither is there a duty to proof a professional training or ability for the business. Real estate agencies do not have a good reputation in the Czech Republic and with many citizens they are unfavourable. As a reaction upon contemporary unsatisfactory situation, the Economic Bureau of the Czech Chamber of Deputies elected an operative group that is supposed to maintain a survey of the contemporary situation in the real estate market and suggest possible solutions. This work aims also to analyse the suggested solutions and to supply solutions that would be missing.