Iron and Diabetes Risk, Judith A. Simcox, Donald A. McClain; Cell Metabolism, Volume 17, Issue 3, 5 March 2013, Pages 329-341, http://dx.doi.org/10.1016/j.cmet.2013.02.007
Friedreich ataxia is associated with increased prevalence of type 2 diabetes. Abnormal IRP1 activity is seen in patients with Friedreich ataxia. It's thought frataxin is an iron chaperone or storage protein. IRP1 aconitase activity is decreased, while IRP binding to IREs is increased.
Tuesday, March 5, 2013
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).
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).
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