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Molecular genetic analysis of patients with Usher syndrome
Průšová, Kateřina ; Ďuďáková, Ľubica (advisor) ; Kousal, Bohdan (referee)
The work focuses on molecular genetic testing of patients with Usher syndrome to confirm the diagnosis, to determine the causal cause of the disease and describe new mutations causing Usher syndrome in Czech patients. Usher syndrome is a clinically and genetically heterogeneous disease that is the most common cause of hereditary deafblindness. Based on responsible genes and disease onset is classified into three clinical subtypes. Given the fact that there is currently no specific treatment, there is a need to understand the pathophysiology of this disease and to broaden the spectrum of causal mutations. The theoretical part of the thesis deals with the anatomy of the eye, especially the structure of the retina. Attention is also paid to retinal diseases, such as the progressive loss of vision characteristic for retinitis pigmentosa (RP). RP may occur either as an isolated disorder or also affecting other organs, so-called syndromic RP. Classic syndromic RP includes Usher's syndrome, which the work mainly deals with. The theoretical part of the thesis describes mainly the mechanism of the disease, the functions of individual Usher proteins and the genes that encode these proteins. The haplotype analysis has been previously done for the most common mutations causing Usher's syndrome in Europe Based...
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Book as a Jewel, Fancoflemish Books of Hours from the National Library in Prague
Průšová, Kateřina ; Hlaváčková, Jana Hana (advisor) ; Royt, Jan (referee) ; Panušková, Lenka (referee)
PhDr. Kateřina Průšová Book as a Jewel, Francoflemish Books of Hours from the National Library in Prague Abstract The purpose of this dissertation is to present the first comprehensive catalogue of Franco-Flemish books of hours from the collections of the National Library in Prague (hereafter, NK). Some of the included manuscripts were already published in journals some of them have never been published previously. The core of the catalogue is a formal and stylistic analysis of books of hours, including an attribution to the individual masters and workshops, localization and dating based on a comparison of stylistically related manuscripts. This work is a development from previous my research done on Parisian books of hours [NK Břevnov 297, NK Osek 70 and NK XXIII G 89; Master's thesis and 'rigorous work']. Six books of hours come from the National Library in Prague [NK XXIII H 22, NK Břevnov 296, NK XXIII F 198, NK XXIII G88, NK Osek 65, NK VI D 25] and one manuscript in the collection of the Lobkowicz family at Nelahozeves castle, previously deposited in the Prague National Library [with a shelf- mark VI Fg 67]. The catalogue covers the period of the greatest flowering of Flemish book illumination from the first half of the 15th century to the beginning of the 16th century. The books of hours from the...
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2nd half of the 15th century Parisian illuminated manuscripts in the Prague collections
Průšová, Kateřina ; Royt, Jan (referee) ; Hlaváčková, Jana Hana (advisor)
In the fifteenth century, the production of books of hours was concentrated in the Îlede- France and Burgundy. This specific item was exported more widely from these regions than anywhere else in Europe. The Collections of the Prague National Library include a dozen of French and Flemish books of hours from this epoch. We selected three of Parisian origin for a particular analysis. Interestingly, the selections cover the entire second half of the fifteenth century, which was the pre-eminently plentiful period of parisian illumination. At the turn of the twentieth century, Paul Durrieu collected a large group of manuscripts that he attributed to Jacques de Besançon, after a colophone in one parisian missal (Bibliothque Mazarine, Ms. 461). Later, when Thuasne published Gauguin's letter to Charles de Gaucourt speaking about "egregius pictor Franciscus", the artist was rebaptised on Maître François. However, this illuminator was not considered to be the creator of this large group for long. As soon as the 1930's, Eleanor P. Spencer started to work on the manuscript collection and illucidated the three successive workshops in Paris of the second half of the 15th century; first, Master of Jean Rolin II, then Maître François and, finally, the Chief Associate of Maître François or Maître de Jacques de Besançon....
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