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Kucerova 2017 Thesis

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Publications in the MiPMap
Kučerová S (2017) Monitoring of the development of the Huntignton´s disease in transgenic minipigs with N-terminal part of human mutated huntingtin: biochemical and motoric changes of F0, F1 and F2 generation. Diploma Thesis p85.

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Kucerova S (2017) Diploma Thesis

Abstract: Huntington´s disease (HD) belongs to neurodegenerative disorders. It is a monogenic disease caused by trinucleotic CAG expansion in exon 1 of gene coding protein huntingtin. Even though the cause of HD is known since 1993, the pathophysiology and cure for HD remains to be found. Animal models are being used for better understanding of HD. The most common animal models for HD are rodents, especially mice but it was also important to create large animal models, which will be more like human. Therefore, the TgHD minipig was created in the Academic of Science in Liběchov, in 2009. This model was created by microinjection of a lentiviral vector carrying the N-terminal part of human HTT with 124 repetitive CAG in exon 1. This model is viable and in every generation, is part of the offspring transgenic.

In this thesis, I specialized to biochemical and behavioral changes of this model. I compared transgenic and wild type siblings. I found that biochemical changes are manifested mostly by an increased level of mtHtt fragments in testes and brain. In the behavioral part of this thesis I established new methods for testing behavioral changes in this model. The introduced methods showed some changes between wild type and transgenic animals at the tested ages but these changes were not significant due to the low number of animals at the oldest age. Nevertheless, these methods will be further used in aging animals to compare the phenotype development in this model with well-known behavioral and motoric changes typical for HD patients. Keywords: Huntington´s disease, Huntingtin, Animal model, Miniature pig, Behavioral studies Bioblast editor: Kandolf G


Labels: Pathology: Neurodegenerative 

Organism: Pig