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Molecular Basis of Hereditary Hyperuricaemic Nephropathies
Vyleťal, Petr ; Kmoch, Stanislav (advisor) ; Viklický, Ondřej (referee) ; Jirsa, Milan (referee)
Familial juvenile hyperuricemic nephropathy (FJHN) and medullary cystic kidney disease type 1 (MCKD1) and type 2 (MCKD2) are autosomal dominant tubulointerstitial nephropathies characterized by combinations of hyperuricaemia, gouty arthritis, progressive renal insufficiency, and in some but not all families, medullary cysts. The phenotypic expression of these diseases is inconsistent, overlaps and indicates broader genetic and allelic heterogeneity. Their pathophysiology was mainly unknown. Previous studies localized FJHN/MCKD genes to chromosomes 16p11 and 1q21. This thesis was primarily aimed at identification of molecular bases and mechanisms underlying FJHN/MCKD. To follow this aim, we focused on collection and characterization of FJHN/MCKD patients and families, identification of disease causing genes in affected families, characterisation of identified proteins and their mutated forms and the isolation and characterisation of interacting partners of newly identified proteins. We employed and established numerous molecular genetic, molecular biological and biochemical methods. We gathered one of the largest sets of families with FJHN/MCKD in the world. In about 26% of families we identified UMOD (uromodulin encoding) gene mutations and characterised by various approaches 6 uromodulin mutant proteins....
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Escape from the chains of sense: towards the subversive force of the texts of F. M. Dostojevsky, F. Kafka and W. Gombrowicz
Jirsa, Tomáš ; Heczková, Libuše (advisor) ; Málek, Petr (referee)
My thesis "Escape from the Chains of Sense: Towards the Subversive Force of the Texts of F. M. Dostoevsky, F. Kafka and W. Gombrowicz" focuses on the phenomenon here called the f1ight of sense. This notion challenges interpretation as a desire for order and for synthesis of the work's semantic direction, and caUs for a different reading of the work. In the introduction, using Adorno's, Deleuze's and Guattari' s concepts, I try to show that the f1ight of sense is not a final state (being without sense) but the act of its f1eeing. Inf1uenced by Barthes and his concept of the pleasure ofthe text, I call the spaces ofthe subversive force, which lets the sense and its reconstruction slip away, ruptures. They lead to a "creative rnisunderstanding" which is a ground for experirnentation. This experimentation does not refuse interpretation and its hermeneutic claim on clear understanding but suggests a different approach: a reading that looks through these ruptures and lets itself get seduced by the gestures and ornaments, which defer or dissolve meaning in the text s of Do sto evsky, Kafka and Gombrowicz. Thus experimentation is an attempt to describe how these elements work together.
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Preparation of a hearing in the civil procedure
Jirsa, Martin ; Sedláček, Miroslav (advisor) ; Smolík, Petr (referee)
Preparation of a hearing in the civil procedure The topic of this thesis is preparation of a hearing in civil procedure. It represents a phase of civil procedure which starts the moment the action it brought to the court and ends with the hearing itself. The hearing should be regarded as the culmination of the whole procedure, which is why the right approach to the preparation of a hearing is not any less important than the hearing itself. The aim of this thesis is to give a comprehensive overview of this issue by reviewing the past, analysing the present legislation and providing de lege ferenda reflection. The thesis is divided into three parts. The first one is a historical overview, in which the author describes the legal development of the preparation of a hearing since 19th century until present. Legal principles, that has formed during this legal evolution and that are affecting today's legislation, are described in the beginning of the second part of the thesis. Principle of concentration and of single hearing being the two most emphasized principles. The majority of the second part deals with the current legislation, which is primarily contained in the art. 114 to 114c of the Czech Civil Procedure Code. The thesis describes the specific steps that the court has to undertake after the...
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Transcending egoism through utilitarianism
Vaškovic, Petr ; Jirsa, Jakub (advisor) ; Hill, James (referee)
This bachelor's thesis transcending egoism through utilitarianism deals with the problem of egoism in connection to moral acts, especially in connection to the motivation of moral action. Main focus of the thesis is on the problem of egoism, which seems to be one of the main motivating powers, usually negative, that is preventing individual from following his moral judgment. Transcending the negative influence of egoism is possible through adopting a certain impersonal viewpoint. This tendency, namely the aspiration to achieve an impersonal viewpoint can be found in utilitarianism. Utilitarianism in its foundations works with an Epicurean, therefore more or less egoistically hedonistic view of morality, with a tendency of an individual to follow pleasure and avoid pain. Impersonal character of utilitarianism, that aspect of utilitarianism, which is capable of transcending egoism, is the aspect which will be most important for this thesis. The ability of utilitarianism to transcend the personal viewpoint will be the core of the argument, which will help us in transcending egoism. Keywords Ethics, egoism, motivation, utilitarianism, Bentham, Mill, Scheffler, Hare
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The void in the Epicureism
Tříletý, Petr ; Thein, Karel (advisor) ; Jirsa, Jakub (referee)
The essay deals with the role of void in the Epicurean physics and asks two fundamental questions: What was void for Epicurus and why had he thought it must exist. As a reply to the first question two conceptions are possible - that the void means emptiness betweeen atoms and that it means the space in general. Arguments are given for the claim that Epicurus operated both these concepts. The first one, an empty interval, is necessary for the possibility of motion in atomism and a wider notion of the space, among other reasons, seems to be too important for speaking about the first one. The second question focuses on reasons for existence of the void. Atomists were convinced that it is necessary condition of possibility of locomotion. But why? There clearly is another possible explanation - motion could be enabled by fluid, matter the speciality of which is ability to change shape. However, examination of fluidity shows that it is provided by infinite divisibility of matter. The atomists' rejection of infinite divisibility then can be understood as their reason for rejecting possibility of fluid matter and thus for needing the void to explain motion.
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Escape from the chains of sense: towards the subversive force of the texts of F. M. Dostojevsky, F. Kafka and W. Gombrowicz
Jirsa, Tomáš ; Málek, Petr (referee) ; Heczková, Libuše (advisor)
My thesis "Escape from the Chains of Sense: Towards the Subversive Force of the Texts of F. M. Dostoevsky, F. Kafka and W. Gombrowicz" focuses on the phenomenon here called the f1ight of sense. This notion challenges interpretation as a desire for order and for synthesis of the work's semantic direction, and caUs for a different reading of the work. In the introduction, using Adorno's, Deleuze's and Guattari' s concepts, I try to show that the f1ight of sense is not a final state (being without sense) but the act of its f1eeing. Inf1uenced by Barthes and his concept of the pleasure ofthe text, I call the spaces ofthe subversive force, which lets the sense and its reconstruction slip away, ruptures. They lead to a "creative rnisunderstanding" which is a ground for experirnentation. This experimentation does not refuse interpretation and its hermeneutic claim on clear understanding but suggests a different approach: a reading that looks through these ruptures and lets itself get seduced by the gestures and ornaments, which defer or dissolve meaning in the text s of Do sto evsky, Kafka and Gombrowicz. Thus experimentation is an attempt to describe how these elements work together.
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Molecular Basis of Hereditary Hyperuricaemic Nephropathies
Vyleťal, Petr ; Kmoch, Stanislav (advisor) ; Viklický, Ondřej (referee) ; Jirsa, Milan (referee)
Familial juvenile hyperuricemic nephropathy (FJHN) and medullary cystic kidney disease type 1 (MCKD1) and type 2 (MCKD2) are autosomal dominant tubulointerstitial nephropathies characterized by combinations of hyperuricaemia, gouty arthritis, progressive renal insufficiency, and in some but not all families, medullary cysts. The phenotypic expression of these diseases is inconsistent, overlaps and indicates broader genetic and allelic heterogeneity. Their pathophysiology was mainly unknown. Previous studies localized FJHN/MCKD genes to chromosomes 16p11 and 1q21. This thesis was primarily aimed at identification of molecular bases and mechanisms underlying FJHN/MCKD. To follow this aim, we focused on collection and characterization of FJHN/MCKD patients and families, identification of disease causing genes in affected families, characterisation of identified proteins and their mutated forms and the isolation and characterisation of interacting partners of newly identified proteins. We employed and established numerous molecular genetic, molecular biological and biochemical methods. We gathered one of the largest sets of families with FJHN/MCKD in the world. In about 26% of families we identified UMOD (uromodulin encoding) gene mutations and characterised by various approaches 6 uromodulin mutant proteins....
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