This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2009]
Function
2 iron, 2 sulfur cluster binding; ferric iron binding; ferrous iron binding; ferroxidase activity; iron chaperone activity; iron-sulfur cluster binding; protein binding;
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