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Keywords: Mitochondrial function
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Journal Articles

Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease

Open Access
In collection:
Rare Disease Translational Research
Marie Rodinova, Jana Krizova, Hana Stufkova, Bozena Bohuslavova, Georgina Askeland, Zaneta Dosoudilova, Stefan Juhas, Jana Juhasova, Zdenka Ellederova, Jiri Zeman, Lars Eide, Jan Motlik, Hana Hansikova
Journal: Disease Models & Mechanisms
Dis Model Mech (2019) 12 (7): dmm038737.
https://doi.org/10.1242/dmm.038737
Published: 26 July 2019
... be used as potential biomarkers of disease progression and suggest that similar pathogenic mechanisms underlie disease progression in TgHD minipigs and HD patients. Huntington's disease Mitochondrial function Ultrastructure HD large animal model Disease development Skeletal muscle Biomarkers...
View articletitled, Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease
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Journal Articles

A transgenic minipig model of Huntington's disease shows early signs of behavioral and molecular pathologies

Open Access
In collection:
Neurodegenerative Disorders
Georgina Askeland, Marie Rodinova, Hana Štufková, Zaneta Dosoudilova, Monika Baxa, Petra Smatlikova, Bozena Bohuslavova, Jiri Klempir, The Duong Nguyen, Anna Kuśnierczyk, Magnar Bjørås, Arne Klungland, Hana Hansikova, Zdenka Ellederova, Lars Eide
Journal: Disease Models & Mechanisms
Dis Model Mech (2018) 11 (10): dmm035949.
https://doi.org/10.1242/dmm.035949
Published: 24 October 2018
... that a minipig model of Huntington's disease mimics human neurodegeneration and holds promise for future intervention studies. However, minipig peripheral blood mononuclear cells express no detectable mutant huntingtin, eliminating their use as monitoring tools. Huntington's disease Mitochondrial function...
View articletitled, A transgenic minipig model of Huntington's disease shows early signs of behavioral and molecular pathologies
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Includes: Supplementary data