Iron and Copper in Mitochondrial Diseases

Iron and Copper in Mitochondrial Diseases, Wenjing Xu, Tomasa Barrientos, Nancy C. Andrews; Cell Metabolism, Volume 17, Issue 3, 5 March 2013, Pages 319-328


Fe-S cluster biogenesis may not be as severely affected in patients with Friedreich’s ataxia or animals carrying FXN mutations. Mitochondrial Fe accumulation has been reported (Lamarche et al., 1980; Puccio et al., 2001), but others suggest that it is an inconsistent and late finding (Bayot et al., 2011). However, Fxn-deficient mouse cells do show altered expression of genes that favor Fe uptake and mitochondrial import over Fe storage and export (Huang et al., 2009).