National Repository of Grey Literature 5 records found  Search took 0.00 seconds. 
Porcine models for Huntington disease
Růna Vochozková, Petra ; Motlík, Jan (advisor) ; Bohačiaková, Dáša (referee) ; Fulková, Helena (referee)
The causative role of the huntingtin (HTT) gene in Huntington's disease (HD) has been identified more than 25 years ago. The extension of CAG repeat stretch over 39 repeats in exon 1 of one HTT allele results in full penetrance of this neurodegenerative disorder. While the identification of the causative mutation raised hopes that development of the therapeutic compound will be easily achievable, the patients and their families are still waiting for treatment until now. The main reason for that might be the complex cellular function HTT that makes the determination of the pathologic mechanism difficult and the development of treatments even more challenging. Although a lot of different animal models have been generated until now, establishing a suitable model has still not been achieved yet. Due to its anatomy, physiology, and genetics, the minipig seems to be a suitable candidate for neurodegenerative disease models. Indeed, the existing Transgenic (Tg) Libechov minipig model manifests signs typical for HD in patients, but on the other hand significant inconsistencies have also been observed. The finding of malformation that partially shows the situation in human patients is true for both, the male reproductive tract as well as for the brain. The reason for this might be the fact the genetic...
Following the phenotype development of TgHD minipigs by invasive and noninvasive approach
Ellederová, Zdeňka ; Baxa, Monika ; Vidinská, Daniela ; Bohuslavová, Božena ; Vochozková, Petra ; Šmatlíková, Petra ; Klíma, Jiří ; Valeková, Ivona ; Ardan, Taras ; Juhás, Štefan ; Juhásová, Jana ; Konvalinková, R. ; Klempíř, J. ; Pokorný, M. ; Krupička, R. ; Kauler, J. ; Hansíková, H. ; Motlík, Jan
Recent promising treatments for Huntington’s disease (HD) may require pre-clinical testing in large animals. In 2009, we generated HD transgenic (TgHD) minipigs with one copy encoding the N-terminal part (548 aa) of human huntingtin (HTT) with 124 CAG/CAA repeats integrated into chromosome 1 q24-q25. The successful germ line transmission occurred through four successive generations.
Evaluation of strategies for humanization of the entire porcine HTT locus
Vochozková, Petra ; Klymiuk, N. ; Wolf, E. ; Ellederová, Zdeňka ; Motlík, Jan
Because fully suitable large animal models are still lacking for Huntington´s disease, we would like to generate a new minipig model which will have an entirely humanized HTT locus. Given the large size of the HTT gene (approx. 160 kb) we will test two different approaches to humanize the porcine HTT locus in porcine kidney cells (PKCs).
Characterization of immune responses to human polyomavirus MCPyV.
Vochozková, Petra ; Šroller, Vojtěch (advisor) ; Drda Morávková, Alena (referee)
Characterization of immune responses to human polyomavirus MCPyV There have been considerable increase in number of known human polyomaviruses in recent years; currently we register twelve of them. Presumably, majority of human polyomaviruses cause lifelong persistent infection. Primary infection is usually asymptomatic and is followed by appearance of antibodies specific to polyomavirus capsid. Polyomaviruses can cause complication especially in immunocompromised people. Merkel cell polyomavirus (MCPyV) is linked to development o of Merkel cell carcinoma (MCC). Although this skin tumor is very rare, MCPyV infection is very common. Most of the human population exhibit MCPyV-specific antibodies in serum. MCPyV specific antibodies are detected in patients with MCC and their level is generally higher than in healthy individuals. MCC occurs more often in immunosuppressed individuals. It appears that protection of antibodies against tumor formation is not sufficient and the development of the tumor could be rather under the control of cellular immunity. In this study, we have prepared plasmids for production of major capsid protein VP1 and detection of antibodies specific to MCPyV capsids. Mice immunized with DNA vaccine expressing VP1 protein produced VP1 specific antibodies in serum. From insect cells infected...
Human polyomavirus isolated from Merkel cell carcinoma
Vochozková, Petra ; Šroller, Vojtěch (advisor) ; Španielová, Hana (referee)
Human polyomaviruses belong to the Polyomaviridae family. Until now, five human polyomaviruses (BK, JC, KI, WU and MCV) have been discovered. There is described the course of polyomavirus infection in the first part of the thesis. These small nonenveloped viruses penetrate into the host cell by receptor-mediated endocytosis and then travel through the ER pathway to get into the nucleus where the virus replicates and expresses viral proteins. The infection occurs during early childhood and the virus remains asymptomatic in healthy individuals. However, the virus is able to reactivate in the immunosuppressed patients and can cause some diseases. The second part of thesis is focused on the MCV. MCV genom was detected in Merkel cell carcinoma (MCC) two years ago. MCV infects the Merkel cells and its DNA integrates into host cell genome. In most cases, the MCV is detected in cancer cells using PCR. Viral sequences encoding the large and small T antigen were found in the MCC using the same method; moreover, there was expressed a significant amount of oncoproteins. These factors point out an important role of MCV in the tumor progress. The study of MCV may help to discover new approaches for the treatment of MCC and other biologically similar tumors.

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