Number of found documents: 75
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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
2017 - English
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. Keywords: minipigs; Huntington´s disease Available at various institutes of the ASCR
Following the phenotype development of TgHD minipigs by invasive and noninvasive approach

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 ...

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
Ústav živočišné fyziologie a genetiky, 2017

Evaluation of strategies for humanization of the entire porcine HTT locus
Vochozková, Petra; Klymiuk, N.; Wolf, E.; Ellederová, Zdeňka; Motlík, Jan
2017 - English
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). Keywords: minipig model; HTT locus Available at various institutes of the ASCR
Evaluation of strategies for humanization of the entire porcine HTT locus

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 ...

Vochozková, Petra; Klymiuk, N.; Wolf, E.; Ellederová, Zdeňka; Motlík, Jan
Ústav živočišné fyziologie a genetiky, 2017

Stimulating autophagy and reducing inclusions in mouse model of Huntington's disease via expression of transcription factor EB (TFEB)
Vodička, Petr; Chase, K.; Iulliano, M.; Tousley, A.; Valentine, D. T.; Sapp, E.; Kegel-Gleason, K. B.; Sena-Esteves, M.; Aronin, N.; DiFiglia, M.
2017 - English
Mutant huntingtin (mHTT) is encoded by the Huntington’s disease (HD) gene and its accumulation in the brain contributes to HD pathogenesis. Reducing mHTT levels through activation of the autophagosome-lysosomal pathway may have therapeutic benefit. Transcription factor EB (TFEB) regulates lysosome biogenesis and autophagy. We introduced cDNA encoding TFEB with an HA tag (TFEB-HA) under the control of neuron specific synapsin 1 promoter into the striatum of 3 month old HDQ175/Q7 mice using adeno-associated virus AAV2/9. Keywords: mutant huntingtin Available at various institutes of the ASCR
Stimulating autophagy and reducing inclusions in mouse model of Huntington's disease via expression of transcription factor EB (TFEB)

Mutant huntingtin (mHTT) is encoded by the Huntington’s disease (HD) gene and its accumulation in the brain contributes to HD pathogenesis. Reducing mHTT levels through activation of the ...

Vodička, Petr; Chase, K.; Iulliano, M.; Tousley, A.; Valentine, D. T.; Sapp, E.; Kegel-Gleason, K. B.; Sena-Esteves, M.; Aronin, N.; DiFiglia, M.
Ústav živočišné fyziologie a genetiky, 2017

Body fluid exosomes as potential carriers of Huntington’s disease biomarkers
Kupcová Skalníková, Helena; Červenka, Jakub; Bohuslavová, Božena; Turnovcová, Karolína; Vodička, Petr
2017 - English
Huntington’s disease (HD) is a hereditary neurodegenerative disorder characterized by a progressive motor, behavioural, and cognitive decline, ending in death. The cause of HD is an abnormal expansion of CAG repeats in HTT gene resulting in prolonged polyglutamine (polyQ) sequence in huntingtin protein (HTT). Huntingtin is a large protein (348 kDa) expressed ubiquitously through the body, with highest expression in the brain and testes. To study HD pathophysiology and to test experimental therapies, a transgenic HD minipig (TgHD) model expressing N-terminal part (N548-124Q) of human mutated huntingtin (mHTT) under the control of human huntingtin promoter was created in Libechov. Beside the mild neurological impairment, the TgHD boars show decreased fertility after 13th month of age. Keywords: Huntington´s disease Available at various institutes of the ASCR
Body fluid exosomes as potential carriers of Huntington’s disease biomarkers

Huntington’s disease (HD) is a hereditary neurodegenerative disorder characterized by a progressive motor, behavioural, and cognitive decline, ending in death. The cause of HD is an abnormal expansion ...

Kupcová Skalníková, Helena; Červenka, Jakub; Bohuslavová, Božena; Turnovcová, Karolína; Vodička, Petr
Ústav živočišné fyziologie a genetiky, 2017

The effect of WNT5A and WNT7A on cartilage formation and anterior-posterior patterning of the limb
Killinger, Michael; Buchtová, Marcela
2017 - English
Growth and patterning of the vertebrate limb is controlled by signals produced in the appical ectodermal ridge (AER) and by the zone of polarizing aktivity (ZPA). limb development requires tight interactions between these signalling centers to keep asymmetrical arrangement of all skeletal elements and their growth. Keywords: limb Available at various institutes of the ASCR
The effect of WNT5A and WNT7A on cartilage formation and anterior-posterior patterning of the limb

Growth and patterning of the vertebrate limb is controlled by signals produced in the appical ectodermal ridge (AER) and by the zone of polarizing aktivity (ZPA). limb development requires tight ...

Killinger, Michael; Buchtová, Marcela
Ústav živočišné fyziologie a genetiky, 2017

Potent anti-spasticity and anti-nociceptive effect of subpial GAD65 and VGAT gene delivery in rat and mice
Maršala, M.; Tadokoro, T.; Hernandez, M. B.; Navarro, M.; Maršala, S.; Miyanohara, A.; Juhás, Štefan; Juhásová, Jana; Platoshyn, O.
2017 - English
In previous studies we have developed a novel spinal subpial (SP) AAV delivery technique in adult mice, rats and minipigs. Using this technique we have demonstrated potent and wide-spread transgene (GFP) expression in spinal white and gray matter after a single SP bolus of AAV9-UBI-GFP. Keywords: rat; mouse Available at various institutes of the ASCR
Potent anti-spasticity and anti-nociceptive effect of subpial GAD65 and VGAT gene delivery in rat and mice

In previous studies we have developed a novel spinal subpial (SP) AAV delivery technique in adult mice, rats and minipigs. Using this technique we have demonstrated potent and wide-spread transgene ...

Maršala, M.; Tadokoro, T.; Hernandez, M. B.; Navarro, M.; Maršala, S.; Miyanohara, A.; Juhás, Štefan; Juhásová, Jana; Platoshyn, O.
Ústav živočišné fyziologie a genetiky, 2017

Produkce bioplynu a mikrobiální profil bioplynové stanice
Fliegerová, Kateřina; Mrázek, Jakub; Štrosová, Lenka; Bubíková, Hana; Křepelková, M.; Hašek, L.; Erban, I.; Lhota, T.
2014 - Czech
Keywords: biogas Available at various institutes of the ASCR
Produkce bioplynu a mikrobiální profil bioplynové stanice

Fliegerová, Kateřina; Mrázek, Jakub; Štrosová, Lenka; Bubíková, Hana; Křepelková, M.; Hašek, L.; Erban, I.; Lhota, T.
Ústav živočišné fyziologie a genetiky, 2014

E-INFRASTRUCTURES FOR RESEARCH AND INNOVATION: LINKING INFORMATION SYSTEMS TO IMPROVE SCIENTIFIC KNOWLEDGE PRODUCTION
Ráb, Petr
2012 - English
The workshop gives an overview about CRIS in the Czech Republic and Slovakia. It consists of a number of short presentations that mention important aspects and current question about CRIS in the two neighbouring countries. Research evaluation is among the important topics as well. Keywords: CRIS Available at various institutes of the ASCR
E-INFRASTRUCTURES FOR RESEARCH AND INNOVATION: LINKING INFORMATION SYSTEMS TO IMPROVE SCIENTIFIC KNOWLEDGE PRODUCTION

The workshop gives an overview about CRIS in the Czech Republic and Slovakia. It consists of a number of short presentations that mention important aspects and current question about CRIS in the two ...

Ráb, Petr
Ústav živočišné fyziologie a genetiky, 2012

Vliv dlouhodbého podávání Ovosanu na hematologické parametry a zastoupení imunitních buněk u onkologických pacientů
Horák, Vratislav; Holubová, Monika; Pokorná, E.
2011 - Czech
Přes rozsáhlý výzkum a nové terapeutické postupy představují nádorová onemocnění druhou nejčastější příčinu umrtí. Podle webového portálu "Epidemiologie zhoubných nádorů v České republice", který zpracovává údaje Národního onkologického registru od roku 1977 pomocí softwaru SVOD (http://www.svod.cz)je zřejmé, že u některých diagnóz (např. karcinom pankreatu) je v tomto období patrný výrazný nárůst incidence i mortality. Despite extensive research and new therapeutic approaches are cancer diseases the second most common cause of death. According to the website, "Epidemiology of malignant tumors in the Czech Republic", which processes data from the National Cancer Registry in 1977 by software SVOD (http://www.svod.cz) it is clear that for some diagnoses (eg pancreatic cancer) is this period, a significant increase in incidence and mortality. Keywords: Ovosan; oncology Available in a digital repository NRGL
Vliv dlouhodbého podávání Ovosanu na hematologické parametry a zastoupení imunitních buněk u onkologických pacientů

Přes rozsáhlý výzkum a nové terapeutické postupy představují nádorová onemocnění druhou nejčastější příčinu umrtí. Podle webového portálu "Epidemiologie zhoubných nádorů v České republice", který ...

Horák, Vratislav; Holubová, Monika; Pokorná, E.
Ústav živočišné fyziologie a genetiky, 2011

Informační systémy využívané pro současné hodnocení činnosti pracovišť AV ČR za roky 2005-2009
Ráb, Petr; Laiblová Kadlecová, Ivana; Horníček, L.
2011 - Czech
Vedení AV ČR organizuje od roku 1993 pravidelná hodnocení svých pracovišť. V dubnu 2011 skončila první fáze 4. řádného kola hodnocení za období 2005–2009. Současný evaluační proces využívá dva systémy, ASEP/ARL jako podklady pro bibliometrickou a on-line informační systém pro panelovou část hodnocení. Náš příspěvek se zabývá podrobným popisem těchto dvou systémů a na příkladech ukazuje jeho použití při evaluaci pracovišť AV ČR Leadership Academy of Sciences since 1993, organizes regular assessments of their workplaces. In April 2011 ended the first phase of the fourth round proper evaluation for the period 2005-2009. The current evaluation process uses two systems, ASEP / ARL as a basis for bibliometric and on-line information system for the longitudinal part of the evaluation. Our contribution deals with the detailed description of these two systems and shows examples of its use in the evaluation of the AS CR Keywords: Information systems; evaluation of the AS CR Available in a digital repository NRGL
Informační systémy využívané pro současné hodnocení činnosti pracovišť AV ČR za roky 2005-2009

Vedení AV ČR organizuje od roku 1993 pravidelná hodnocení svých pracovišť. V dubnu 2011 skončila první fáze 4. řádného kola hodnocení za období 2005–2009. Současný evaluační proces využívá dva ...

Ráb, Petr; Laiblová Kadlecová, Ivana; Horníček, L.
Ústav živočišné fyziologie a genetiky, 2011

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