Number of found documents: 89
Published from to

Establishing preclinical proof-of-concept of gene therapy for Huntington disease
Miniariková, J.; Juhás, Štefan; Caron, N.; Spronck, L.; Vallés, A.; De Haan, M.; Blits, B.; Ellederová, Zdeňka; van Deventer, S.; Petry, H.; Southwell, A.; Déglon, N.; Motlík, Jan; Konstantinová, P.; Evers, M.
2017 - English
Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the HTT gene. The translated expanded polyglutamine repeat in the huntingtin protein is known to cause toxic gain-of-function, affecting numerous cellular processes. Our approach involves a new therapeutic modality by developing a single (one-time) treatment for HD based on a gene therapy lowering the expression of the toxic huntingtin using the RNA interference (RNAi) mechanism. Huntingtin lowering is achieved using gene transfer of a cassette encoding an engineered microRNA targeting human HTT, delivered via adeno-associated viral vector serotype 5 (AAV5-miHTT). Keywords: Huntington´s disease Available at various institutes of the ASCR
Establishing preclinical proof-of-concept of gene therapy for Huntington disease

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the HTT gene. The translated expanded polyglutamine repeat in the ...

Miniariková, J.; Juhás, Štefan; Caron, N.; Spronck, L.; Vallés, A.; De Haan, M.; Blits, B.; Ellederová, Zdeňka; van Deventer, S.; Petry, H.; Southwell, A.; Déglon, N.; Motlík, Jan; Konstantinová, P.; Evers, M.
Ústav živočišné fyziologie a genetiky, 2017

Oxidative stress in primary porcine fibroblasts expressing mutated huntingtin
Šmatlíková, Petra; Askeland, G.; Vaškovičová, Michaela; Klíma, Jiří; Motlík, Jan; Eide, L.; Ellederová, Zdeňka
2017 - English
Molecular events, such as protein aggregation, mitochondrial dysfunction, and transcriptional dysregulation have been linked to Huntington’s disease (HD) pathogenesis. Oxidative stress has been considered as one of the key players in disease progression. Though, it is still not clear whether oxidative stress causes HD, or if it is a consequence of other primary events. Keywords: huntingtin Available at various institutes of the ASCR
Oxidative stress in primary porcine fibroblasts expressing mutated huntingtin

Molecular events, such as protein aggregation, mitochondrial dysfunction, and transcriptional dysregulation have been linked to Huntington’s disease (HD) pathogenesis. Oxidative stress has been ...

Šmatlíková, Petra; Askeland, G.; Vaškovičová, Michaela; Klíma, Jiří; Motlík, Jan; Eide, L.; Ellederová, Zdeňka
Ústav živočišné fyziologie a genetiky, 2017

Results of the realisation of the experiments using large experimental animals – application of cells/ stem cells using different vectors, diagnostics
Juhás, Štefan; Juhásová, Jana
2017 - English
A design of methods to test application of cells/ stem cells using different vectors, solution of the application including diagnostics and connected agenda. Keywords: large animal models; stem cell therapy; spinal stem cells Available at various institutes of the ASCR
Results of the realisation of the experiments using large experimental animals – application of cells/ stem cells using different vectors, diagnostics

A design of methods to test application of cells/ stem cells using different vectors, solution of the application including diagnostics and connected agenda.

Juhás, Štefan; Juhásová, Jana
Ústav živočišné fyziologie a genetiky, 2017

Výsledky kurzu in vivo IBD na minipraseti
Juhás, Štefan; Juhásová, Jana
2017 - Czech
Testování inovativních postupů léčby IBD na miniaturních prasatech. Innovative approaches testing of Inflammatory Bowel Disease on minipigs. Keywords: minipig; IBD; operational equipment Available at various institutes of the ASCR
Výsledky kurzu in vivo IBD na minipraseti

Testování inovativních postupů léčby IBD na miniaturních prasatech.


Innovative approaches testing of Inflammatory Bowel Disease on minipigs.

Juhás, Štefan; Juhásová, Jana
Ústav živočišné fyziologie a genetiky, 2017

Realizace pokusů na laboratorních zvířatech
Juhás, Štefan; Juhásová, Jana
2017 - Czech
Návrh způsobu ověření funkčnosti inovativních náhrad, řešení tohoto návrhu včetně implantací a souvisejících aktivit. A design of methods to test a functionality of inovative replacements, solution of the replacement incl. implantation and connected agenda. Keywords: implants; experimental animals Available at various institutes of the ASCR
Realizace pokusů na laboratorních zvířatech

Návrh způsobu ověření funkčnosti inovativních náhrad, řešení tohoto návrhu včetně implantací a souvisejících aktivit....

Juhás, Štefan; Juhásová, Jana
Ústav živočišné fyziologie a genetiky, 2017

Výzkum v oboru oftalmologie, implantologie a buněčné biologie
Ardan, Taras
2017 - Czech
Výzkum v oblasti reakce organismu a specifických buněk oka na inovativní implantát vyvinutý objednatelem. Research on the reaction of the organism and specific eye cells on the innovative implant developer by the contractor. Keywords: intraocular; cells; implant Available at various institutes of the ASCR
Výzkum v oboru oftalmologie, implantologie a buněčné biologie

Výzkum v oblasti reakce organismu a specifických buněk oka na inovativní implantát vyvinutý objednatelem....

Ardan, Taras
Ústav živočišné fyziologie a genetiky, 2017

Role of planar cell polarity pathway in ameloblastoma with focus on frizzled 6
Putnová, Barbora; Putnová, Iveta; Hrubá, Eva; Dosedělová, Hana; Štembírek, Jan; Daněk, Z.; Buchtová, Marcela
2017 - English
Ameloblastoma is the most common epithelial odontogenic tumour in the oral cavity. Its progression is usually slow and the surface is covered by an oral mucosa with physiologic appearance. These tumours do not cause any pain to the patient and therefore it is difficult to diagnose them on time and the first signs are often missed by clinician leading to late diagnosis. Keywords: ameloblastoma Available at various institutes of the ASCR
Role of planar cell polarity pathway in ameloblastoma with focus on frizzled 6

Ameloblastoma is the most common epithelial odontogenic tumour in the oral cavity. Its progression is usually slow and the surface is covered by an oral mucosa with physiologic appearance. These ...

Putnová, Barbora; Putnová, Iveta; Hrubá, Eva; Dosedělová, Hana; Štembírek, Jan; Daněk, Z.; Buchtová, Marcela
Ústav živočišné fyziologie a genetiky, 2017

Role of FGF signaling in zeugopod development
Buchtová, Marcela; Celá, Petra; Horáková, D.; Krejčí, P.
2017 - English
The fibroblast growth factor (FGF) signalling systém regulates many developmental processes including skeletogenesis, brain patterning, branching morphogenesis or limb development. as FGFs are key players in the variable processes of chondrogenesis, we experimentally manipulated this pathway to test its effect on zeugopode modeling. Keywords: FGF Available at various institutes of the ASCR
Role of FGF signaling in zeugopod development

The fibroblast growth factor (FGF) signalling systém regulates many developmental processes including skeletogenesis, brain patterning, branching morphogenesis or limb development. as FGFs are key ...

Buchtová, Marcela; Celá, Petra; Horáková, D.; Krejčí, P.
Ústav živočišné fyziologie a genetiky, 2017

Mechanism of complex tooth shape development in reptiles
Landová, Marie; Zahradníček, Oldřich; Dosedělová, Hana; Kavková, M.; Zikmund, T.; Buchtová, Marcela
2017 - English
Tooth shape formation in mammals is well known proces thanks to the broad studies on the mouse molars. The main role in regulation of this proces plays the enamel knot. Enamel knots are formed by cluster of cells, which serves as a source of numerous signalling molecules such as SHH or members of WNT, BMP and FGF families. Almost all our knowledge about teeth came from study on the mouse model. For better understanding of odontogenesis, it is however necessary to compare developmental processes to another species. One of the most heterogenic group of dentition type and shape are reptiles. How the shape of their teeth completed is still unknown. Aim of our study is therefore to uncover developmental processes involved in cusp formation in non-mammalian species with focus on reptiles. As model species for this study, we selected chameleons where multicuspis teeth develop in the caudal area of the jaw. Keywords: reptiles; tooth shape development Available at various institutes of the ASCR
Mechanism of complex tooth shape development in reptiles

Tooth shape formation in mammals is well known proces thanks to the broad studies on the mouse molars. The main role in regulation of this proces plays the enamel knot. Enamel knots are formed by ...

Landová, Marie; Zahradníček, Oldřich; Dosedělová, Hana; Kavková, M.; Zikmund, T.; Buchtová, Marcela
Ústav živočišné fyziologie a genetiky, 2017

Mitochondrial phenotype in minipig model transgenic for N-terminal part of human mutated huntingtin
Hansíková, H.; Rodinová, M.; Křížová, J.; Dosoudilová, Z.; Štufková, H.; Bohuslavová, Božena; Klíma, Jiří; Juhás, Štefan; Ellederová, Zdeňka; Motlík, Jan; Zeman, J.
2017 - English
Huntington’s disease (HD) is neurodegenerative disorder caused by an abnormal expansion of CAG repeat encoding a polyglutamine tract of huntingtin (htt). It has been postulated that mitochondria dysfunction may play significant role in the pathophysiology of the HD. But it is still not known yet in detail how mitochondria are able to cover energy needs of the cells during the progression of the HD. Keywords: minipig model; Huntington´s disease Available at various institutes of the ASCR
Mitochondrial phenotype in minipig model transgenic for N-terminal part of human mutated huntingtin

Huntington’s disease (HD) is neurodegenerative disorder caused by an abnormal expansion of CAG repeat encoding a polyglutamine tract of huntingtin (htt). It has been postulated that mitochondria ...

Hansíková, H.; Rodinová, M.; Křížová, J.; Dosoudilová, Z.; Štufková, H.; Bohuslavová, Božena; Klíma, Jiří; Juhás, Štefan; Ellederová, Zdeňka; Motlík, Jan; Zeman, J.
Ústav živočišné fyziologie a genetiky, 2017

About project

NRGL provides central access to information on grey literature produced in the Czech Republic in the fields of science, research and education. You can find more information about grey literature and NRGL at service web

Send your suggestions and comments to nusl@techlib.cz

Provider

http://www.techlib.cz

Facebook

Other bases