Iron-induced oligomerization of human FXN 81-210 and bacterial CyaY frataxin and the effect of iron chelators

Eva-Christina Ahlgren, Mostafa Fekry, Mathias Wiemann, Christopher A. Söderberg, Katja Bernfur, Olex Gakh, Morten Rasmussen, Peter Højrup, Cecilia Emanuelsson, Grazia Isaya, Salam Al-Karadaghi. PLoS ONE 12(12): e0188937. doi:10.1371/journal.pone.0188937

Patients suffering from the progressive neurodegenerative disease Friedreich’s ataxia have reduced expression levels of the protein frataxin. Three major isoforms of human frataxin have been identified, FXN42-210, FXN56-210 and FXN81-210, of which FXN81-210 is considered to be the mature form.

 Iron-induced oligomerization of human FXN 81-210 and bacterial CyaY frataxin and the effect of iron chelators