Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function

Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function. Luisa Iommarini, Susana Peralta, Alessandra Torraco, Francisca Diaz, P, Mitochondrion, Available online 29 January 2015, ISSN 1567-7249, http://dx.doi.org/10.1016/j.mito.2015.01.008.


Interestingly, in contrast to what has been observed extensively in clinical studies, the mice did not show any oxidative damage. This difference could be explained because in mice the total deletion of Fxn happens in a certain tissue at a specific time, whereas FRDA is characterized by partial Frataxin deficiency in all cells throughout the lifetime.