Perturbation of cellular proteostasis networks identifies pathways that modulate precursor and intermediate but not mature levels of frataxin

Joseph F. Nabhan, Renea L. Gooch, Eugene L. Piatnitski Chekler, Betsy Pierce & Christine E. Bulawa; (Nature) Scientific Reports 5, Article number: 18251 (2015) doi:10.1038/srep18251

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 Interestingly, a number of treatments caused a change in total amount of FXN protein, without an effect on mature FXN. Our results imply that regulation of FXN protein levels is complex and that total amounts can be modulated chemically and genetically without altering the absolute amount of mature FXN protein.