... 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 Charles University...

... observations that could allow a distinction between causes and secondary effects, and provide a clear link between aging and disease development. To approach this problem, we developed a cellular model in which we can switch off/on in a time-controlled way the frataxin gene partially mimicking what happens...