Marian Valko , Klaudia Jomova, Christopher J. Rhodes, Kamil Kuča, Kamil Musílek; Archives of Toxicology, First online: 07 September 2015 DOI:10.1007/s00204-015-1579-5
Frataxin is a mitochondrial iron chaperone protein and plays a key role in the insertion of ferrous ions during the assembly of iron–sulfur clusters in the mitochondrial respiratory chain. The defect leads to the release of mitochondrial iron with in turn may catalyze hydroxyl radical formation via Fenton chemistry.
Source: Redox- and non-redox-metal-induced formation of free radicals and their role in human disease