Since mitochondrial complex activities of cardiac fibers from FRDAkD mice were impaired without changes in mitochondrial mass, we hypothesize that bioenergetic changes could be attributable to altered fragmentation of the mitochondrial network. This is the first set of data providing ex vivo evidence of mitochondrial respiratory defects during FXN knockdown associated with FRDA. Furthermore, these findings highlight that there are tissue-specific mechanisms during FXN loss since cardiac and skeletal muscle fibers had differential bioenergetic responses.
Monday, January 30, 2023
Decreased mitochondrial respiration in cardiac fibers isolated from a mouse model of Friedreich’s ataxia
PT Pierce, X Wang, A Rivera, A Cooper, H Van Remmen, J Brown, PF Vitiello, The American Journal of the Medical Sciences, Volume 365, Supplement 1, 2023, Pages S194-S195, ISSN 0002-9629, doi:10.1016/S0002-9629(23)00372-5.
