National Repository of Grey Literature 19 records found  1 - 10next  jump to record: Search took 0.00 seconds. 
Phenotypic Analyses of the HD Transgenic Minipig Model (A11609)
Ellederová, Zdeňka
The transgenic Huntington's disease minipigs (TgHD) express N‐terminal part of human mutated huntingtin (124Q) under the control of human huntingtin promoter. The founder animal, born in 2009, gave birth to four subsequent generations with an equal contribution of wild‐type (WT) and transgenic (TgHD) piglets in all litters. The model is being used for preclinical huntingtin lowering studies. Here we take different non-invasive and invasive approaches, some of which are unique for large animal models, to study the phenotype development comparing WT and TgHD siblings. We show gradual progression of the disease in these TgHD animals. Moreover, some biomarkers were identified. These markers could serve for monitoring of organism response to HD treatment to assess efficacy and safety in preclinical studies prior to human clinical trials.
Results of nanomaterials testing for skin wounds nursing
Juhás, Štefan ; Juhásová, Jana
Using minipigs experiments, we tested the healing of fresh wounds treated with different nanofibrous materials and compared them with standard treatment. From predefined wounds, samples were taken at regular intervals for histological analysis, microbiology, wound measurements and photodocumentation. Blood was also collected from the miniprasate to determine pro-inflammatory cytokines.
Results of in vivo nanomaterials testing using minipig
Juhás, Štefan ; Juhásová, Jana
Using in vivo experiments we tested healing of a fresh wound treated with a nanofibrous cover. From predefined wounds, samples were taken at regular intervals for histological analysis, wound measurements and photodocumentation were performed.
Written report of the contractor on the results of the research in ophtalmology, implantation and cell biology.
Ardan, Taras
Research on the reaction of the organism and specific eye cells on the innovative implant developer by the contractor.
Results of the realisation of the experiments using the experimental animals
Juhás, Štefan ; Juhásová, Jana
A design of methods to test a functionality of inovative replacements, solution of the replacement incl. implantation and connected agenda.
Implant study protocol including results
Juhás, Štefan ; Juhásová, Jana
Testing and analysis of implant material and design in real environment.
In vivo IBD using minipig course results
Juhás, Štefan ; Juhásová, Jana
Innovative approaches testing of Inflammatory Bowel Disease on minipigs.
Results of the realisation of the experiments using large experimental animals – application of cells/ stem cells using different vectors, diagnostics
Juhás, Štefan ; Juhásová, Jana
A design of methods to test application of cells/ stem cells using different vectors, solution of the application including diagnostics and connected agenda.
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.
Double strand DNA breaks response in Huntington´s disease transgenic minipigs
Vaškovičová, Michaela ; Šmatlíková, Petra ; Herbert, A. ; Motlík, Jan ; Šolc, Petr
Huntington’s disease (HD) is progressive neurodegenerative disorder caused by presence of CAG expansion in the huntingtin gene, which gives rise to mutated form of huntingtin protein (mHtt). There is a strong evidence that DNA damage response is compromised by presence of mHtt in cells and increase of double strand DNA breaks (DSBs) is an early event in HD pathology. It was shown, that level of γH2AX is significantly higher in R6/2 mice compared to wild-type animals. Moreover, level of γH2AX is higher also in striatal neurons and fibroblasts of human HD patients. Furthermore, protein p53, key player in DNA damage response, is hyperactivated in cells expressing mHtt and inhibition of p53 or ATM ameliorates phenotypes of HD animal models. However, exact mechanism of mHtt action is not clear and therefore further investigation of mHtt effects on DSBs response is very important for the understanding of HD pathology.

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