Huntington's disease (HD) is a neurodegenerative disease with cognitive, motor and psychiatric symptoms. A toxic accumulation of misfolded mutant huntingtin protein (Htt) induces mitochondrial dysfunction, leading to a bioenergetic insufficiency in neuronal and muscle cells. Improving mitochondrial function has been proposed as an opportunity to treat HD, but it is not known how mitochondrial function in different tissues relates.
Brain Bio-Energetic State Does Not Correlate to Muscle Mitochondrial Function in Huntington's Disease.
CHDR
van Diemen MPJ, Hart EP, Hameeteman PW, Coppen EM, Winder JY, den Heijer J, Moerland M, Kan H, van der Grond J, Webb A, Roos RAC, Groeneveld GJ
