Figure 5
Levels of mature frataxin and isoform E in whole blood. Mature frataxin and isoform E levels in whole blood from healthy donors with GAA repeats < 40 were determined by stable isotope dilution LC-MS. Shown are the mean value ± standard deviation (n = 10). The variability in the amount of frataxin isoform E in erythrocytes compared to mature frataxin in cells with mitochondria probably arises from its different mechanism of formation through alternative splicing and N-terminal acetylation during translation rather than the normal transcription and translation involved in expression of full-length frataxin.
