Porcine Model of Huntington's Disease [chapter]

Petra Rausova, Petra Vochozkova, Daniela Vidinska, Eva Hrnciarova, Bozena Bohuslavova, Monika Macakova, Ivona Valekova, Stefan Juhas, Taras Ardan, Petr Solc, Jan Motlik, Zdenka Ellederova
2017 Huntington's Disease - Molecular Pathogenesis and Current Models  
At present, we are probably the only research facility to be breeding transgenic Huntington's disease minipigs (TgHD). These minipigs express N-terminal part of human mutated huntingtin including 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. We take different approaches, some of which are unique for large animal models, to
more » ... tudy the phenotype development comparing WT and TgHD siblings. In this chapter, we review these approaches and the phenotype progression in the minipigs. Additionally, we outline perspectives in generation of new models using novel methodology and the potential of pig models in preclinical HD studies.
doi:10.5772/66353 fatcat:6h3ewepj4nem7jiziw7ujyqngu