Thesis (doctoral)--Universität Leipzig, 1909. / Bibliography: p. [v]-vi.

Fabry disease is an inherited defect of lysosomal α-galactosidase A (α-GALA), causing progressive accumulation of glycosphingolipids with terminal α-galactosyl moieties, especially globotriaosylceramide (Gb3Cer) and in to a small extent also galabiosylceramide (Ga2Cer) and blood group B glycolipids, in most tissues and body fluids. This diploma thesis is an extension of previous laboratory studies and intends to contribute to clarification of some specific features of catabolic pathways of glycolipids substrates in lysosomal storage disorders, especially blood group B glycolipids. Therefore, analysis of human pancreas and lungs tissues was performed using TLC imunodetection and immunohistochemical analysis of these glycolipids. The most striking observation was massive accumulation of B-6-2 glycolipid and of others complex B-glycolipids in the pancreas of the patient with Fabry disease with blood group B. The level of blood group B substrates exceeded significantly storage of Gb3Cer substrate. An important part of this work were metabolic experiments in cell cultures in order to answer the question about participation of related glycosidases - α-galactosidase A and α-N- acetylgalactosaminidase (α -NAGA) in the lysosomal degradation of glycosphingolipids with terminal α-galactose. Loading experiments were...

The Master thesis Global talent management in Schindler Company aims to map the concept of the career programme in Schindler Company SCDP (Schindler Career Development Programme) and to assess the feedback of programme participants based on the survey among participants. The thesis is divided into three parts. The first part of the thesis consists of theoretical knowledge on talent management and work with talents. Basic and key terms that are crucial for deeper introduction to the topic are defined in this part as well. The second part already has a practical character and it is dedicated to the program. Its particular phases, objectives and specifics are defined. The research part is based on the survey completed by participants of the programme. The programme is assessed based on the evaluation of the survey and recommendations for its improvement are proposed. The benefit of this thesis is the provision of survey results and recommendations for programme improvements, which can help Schindler with better understanding on their talents´ attitude.

The revitalization of the abandoned industrial complex in the middle of Brněnec (Brünlitz) village. Spaces of the area could become a lively centre not only of the community, but also of our national contemporary history in one place, the place with a powerful story of more than 1100 saved human lives from the Holocaust in the Schindler`s factory – the sub-camp of the concentration camp Gross-Rosen.

Thesis (M.B.A.)--University of Hong Kong, 1997. / Includes bibliographical references.

The main goal of this master thesis is the vast complex of Löw-Beer’s textile factory with a rich history. Areal is located in the central part of the Brněnec. The aim of the thesis is to evaluate the potential of the area. The work in the design accepts history and comes up with ideas on how to bring the life into the complex again and incorporate it into the core of the village. Based on analyzes and consultations, the construction program of existing and proposed buildings was determined. The result of the work is the conversion of factory buildings into a multifunctional complex.

The main goal of the diploma thesis deals with the complex of the former textile factory in Brnenec. This factory complex has a high historical value due to the events that took place here. The buildings of the original textile factory need to be ensure and repaired, as they have fallen into unsustainable conditions over the years due to the owners' inaction. The main idea of the diploma thesis is to create a proposal that connect the original buildings with new creations. As a result, the necessary center of Brněnec will be created with new functions commemorating the history of the place and at the same time contributing to the development of the village and the creation new job opportunities.

Fabry disease (FD) is an X-linked recessive lysosomal storage disorder (LSD) known to affect approximately 1 in every 40,000 males, and a smaller number of females. FD results from a deficiency of functional α-galactosidase (α-GAL), which leads to the accumulation of terminally α-galactosylated substrates in the lysosome. The predominant treatment is Enzyme Replacement Therapy (ERT), requiring the regular infusion of recombinant human α-GAL. More than half of individuals receiving ERT experience a range of adverse infusion reactions, and it has been reported that as many as 88% of patients receiving ERT develop neutralizing IgG antibodies against the drug. In aim of designing a non-immunogenic treatment candidate for Fabry disease ERT, we have engineered the active sites of α-GAL and another homologous family 27 exoglycosylase named α-N-acetylgalactosaminidase (α-NAGAL) to have interconverted substrate specificities. 11 of 13 active site residues are conserved between these two enzymes, and we have shown that their substrate specificities can be interconverted by mutating the two non-conserved active-site residues. We report the kinetic properties of these two mutants along with wild type controls, and use western blotting to show that both mutant enzymes retain their respective wild type enzyme antigenicity. Structural data obtained by X-ray crystallography on the α-GAL mutant (called α-GALSA ) reveals the mechanism by which substrate specificity is dictated between these two proteins, and provides explanations for the mutant’s reduced catalytic efficiency.

This Thesis is a proposal for a prototypical hillside housing community in Los Angeles, California. As a prototype it is responsible for setting an architectural precedent. In this effort, the Thesis continues with focus on issues of construction methodology, urban planning, and land use relationships concerning the present city. Being clear and uncomplicated is the driving force of this architectural process. On the horizon is the 21st Century. Architecture has become increasingly convoluted rather than enlightened. Here, I have focused my attentions on developing a technologically based, material-driven, compassionate solution to answer the issue of housing on the hillsides of Los Angeles. I have realized a clear system of building using uncomplicated technology and material. However, as demonstrated, this system of building provides only an envelope for space definition. It becomes the architectural precedent, a canvas, through which the inhabitant can define his existence. Enlightened limitations. / Master of Architecture

Darbas skirtas EB teisės nuostatų, įtvirtinančių laisvo prekių judėjimo ir paslaugų teikimo laisvės veikimo sąlygas, bei Europos Teisingumo Teismo jurisprudencijos ir EB teisės doktrinos, padedančios atskleisti minėtų laisvių teisinio turinio panašumus ir skirtumus, lyginamajai analizei. Pirmame darbo skyriuje aprašomi laisvo prekių judėjimo ir paslaugų teikimo laisvės prigimtis, tikslas ir turinys, išskiriami pagrindiniai prekės ir paslaugos požymiai, pateikiama Teisingumo Teismo praktikos, iliustruojančios nagrinėjamų laisvių atskyrimo problematiką, pavyzdžiai. Antrame skyriuje analizuojami pagrindiniai Sutarties nuostatų, susijusių su laisvu prekių judėjimu (28 straipsnis) ir paslaugų teikimo laisve (49 straipsnis), taikymo asmenų ir erdvės atžvilgiu principai ir sąlygos. Trečiame darbo skyriuje atskleidžiama minėtų laisvių ribojimo problematika, ES valstybių narių sukurtų kliūčių pobūdis ir apimtis bei su tuo susijusi Teismo jurisprudencijos apžvalga, išskiriant nagrinėjamoms laisvėms bendrus požymius. Ketvirtame skyriuje aptariami EB Sutartyje įtvirtinti bei Teisingumo Teismo suformuluoti ribojimus pateisinantys pagrindai, proporcingumo principas ir jų taikymo mechanizmas. Penktame skyriuje trumpai apibūdinamos vidaus rinkos harmonizavimo priemonės tiek, kiek jos susijusios prekių ir paslaugų judėjimo laisvių įgyvendinimu, konkrečiai Techninių standartų ir reglamentų direktyva (1998 m.) ir Paslaugų direktyva (2006 m.). Šiuo darbu patvirtinama, kad laisvas prekių... [toliau žr. visą tekstą] / This text provides comparative analysis of the EC provisions, establishing the terms of the free movement of goods and freedom to provide services, as well as of jurisprudence of the European Court of Justice and doctrine of EC law, revealing the differences and similarities of the legal content of the relevant freedoms. The first chapter of the text describes the nature, aim and legal content of the free movement of goods and freedom to provide services followed by the examples from the case-law, which illustrate the distinction of the freedoms considered. The second chapter introduces the comparative analysis concerning the conditions of the territorial scope of the EC Treaty provisions on the free movement of goods (Article 28) and freedom to provide services (Article 49) as well as persons bound by the latter rules. The third chapter deals with the topical issues in respect of the restrictions of the relevant freedoms, in particular the nature and scope of the barriers, adopted by the Member States. The fourth chapter of the text focuses on the justification of the freedoms, in particular on the EC Treaty exceptions, judicially created exceptions as well as the principle of proportionality and the application mechanism of the latter in the comparative manner of the freedoms. Finally, the fifth chapter provides the brief introduction of the harmonisation measures as much as they concern with the implementation of the relevant freedoms, particularly Directive on technical... [to full text]