Phenotypic Analyses of the HD Transgenic Minipig Model (A11609)
Ellederová, Zdeňka
2018 - English
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.
Keywords:
minipig
Available at various institutes of the ASCR
Phenotypic Analyses of the HD Transgenic Minipig Model (A11609)
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 ...
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.
Evaluation of efficacy and bio-distribution of AAV5-miHTT in HD minipig brain
Bohuslavová, Božena; Juhás, Štefan; Juhásová, Jana; Ellederová, Zdeňka; Motlík, Jan
2016 - English
Healthy minipigs (n=29) were pre-screened for serum AAV5 neutralizing antibodies (NABs) in serum by uniQure. Conclusions: Intrastriatal/intrathalamic bilateral application of AAV5-CAG-miHtt(3e13/1e13gctotal) or PBS/Sucrose (54μl/216μl) similarly to AA5-CAG-GFP didn´t cause any neurological deficit in transgenic as well as wild type animals. The body weight increased in all animals 84 days after AAV5-CAG-miHtt/PBS-Sucroseintrastriatal/intrathalamic delivery with two exceptions TGanimal L616 (AAV5-CAG-miHtt1e 13g ctotal) and WTanimal L485 (AAV5-CAG-miHtt3e13gc total). The TG boar N176 from group VII (PBS/Sucrose) died during narcosis 56 days after IP/IT application), shortly after CSF and blood collection.The boar breathed heavily and intermittently, then the heart stopped beat and resuscitation failed. We immediately flushed them by 20 L of PBS with heparin and collect all tissues. All animals had normal food consumption. Inflammatory proces in AAV5-CAG-miHtt (1e13 gc total) seems to be similar to PBS/Sucrose injected animals accompanied with no detectable histological pathologies.
Keywords:
minipig; Huntington´s disease
Available at various institutes of the ASCR
Evaluation of efficacy and bio-distribution of AAV5-miHTT in HD minipig brain
Healthy minipigs (n=29) were pre-screened for serum AAV5 neutralizing antibodies (NABs) in serum by uniQure. Conclusions: Intrastriatal/intrathalamic bilateral application of ...
Phenotypic Analyses of the HD transgenic Minipig Model
Motlík, Jan; Ellederová, Zdeňka
2016 - English
The transgenic model of Huntington’s disease in minipig (TgHD) was created in 2009 and information coding the sequence of N-terminal part of human mutated huntingtin was transferred to subsequent generations from both female and male sides. In each litter, transgenic (TgHD) and wild-type (WT) piglets were born in approximately equal ratio. At present, the Laboratory of Cell Regeneration and Plasticity keeps sets of animals in F2 and F3 generations with identical genetic background and bred in identical conditions of feeding and housing. The present research project was focused on a complex of non-invasive and invasive approaches to WT and TgHD minipigs to achieve the entire phenotypic analysis of HD progression in this large animal model.
Keywords:
minipig
Available at various institutes of the ASCR
Phenotypic Analyses of the HD transgenic Minipig Model
The transgenic model of Huntington’s disease in minipig (TgHD) was created in 2009 and information coding the sequence of N-terminal part of human mutated huntingtin was transferred to subsequent ...
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