National Repository of Grey Literature 5 records found  Search took 0.00 seconds. 
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
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.
Protein analysis of selected mitochondrial proteins in the muscle tissue of porcine model of Huntington's disease
Dosoudilová, Žaneta ; Klempíř, Jiří (advisor) ; Baxa, Monika (referee)
Huntington's disease (HD) is an autosomal dominant hereditary neurodegenerative disease characterized by motor, cognitive and behavioral disorders. HD is caused by expansion of CAG triplet (cytosine-adenosine-guanine) located in a gene on the short arm of the fourth chromosome. This expansion encodes an aberrant polyglutamine chain in the protein huntingtin. Physiological and mutated huntingtin (in case of HD) are expressed in almost all tissues and influences many cellular functions. The prevalence of HD in population is about 1 per 10.000. The disease is currently incurable and its mechanisms are not sufficiently understood. Besides affecting the central nervous system HD also affects peripheral tissues, including skeletal muscles. HD disrupts mitochondrial function and damages oxidative phosphorylation system, which has the task of producing energy in the form of ATP in cells. Research of transgenic minipig model for HD could help elucidate the mechanisms of disease's pathogenesis and potential therapeutic strategy. In this diploma thesis, immunodetection with help of specific antibodies to detect changes in amount of 14 selected mitochondrial proteins in skeletal muscle tissue of three age groups of transgenic HD minipigs - 24, 36 and 48 months old was used. Gradual progression in reduced...
Interplay between mutated huntingtin and precancerogenous status in mammalian cells.
Butalová, Nikola ; Baxa, Monika (advisor) ; Koudelková, Lenka (referee)
Huntington's disease is a serious hereditary disorder that causes mortification of neurons. The disease affects individuals around the age of 40. Its characteristics are involuntary movement of the limbs and a progressive dementia. This disorder is currently without any treatment and always ends with patient dying within a period of 15 years after the first symptoms are discovered. Special relation between Huntington's disease and malign neoplasia was observed at the end of the 20th century. This relation shows lower degree of cancer among the patients with this neurodegenerative disorder compared to the general population. An expanded sequence of a CAG section probably protects these persons against advancement of cancer. Creation of an applicable experimental model with characteristic highly resembling human body was necessary for superior research of this disease. This model can be represented by a transgene mini pig carrying a mutated protein huntingtin - tgHD pig. Another model of a mini-pig showing hereditary occurrence of malign lesions - MeLiM pig was created to research cancer. These two specific lines of mini-pigs were crossbred resulting in not only piglets with melanoma and transgene piglets with mtHTT but also transgene piglets having melanoma at the same time. Furthermore the same...
Methodology of „acute“and transgenic Huntington disease model design in miniature pigs and its application for new methods of treatment and drugs testing in neurodegenerative diseases area
Motlík, Jan ; Juhás, Štefan ; Juhásová, Jana ; Vodička, Petr ; Baxa, Monika
The aim of this methodology is to develop a biomedical model of Huntington's disease in miniature pigs and its use in practice for preclinical testing of new treatments both pharmacological and using new molecular biological methods.

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