Iron-mediated inhibition of mitochondrial manganese uptake mediates mitochondrial dysfunction in a mouse model of hemochromatosis.

@article{Jouihan2008IronmediatedIO,
  title={Iron-mediated inhibition of mitochondrial manganese uptake mediates mitochondrial dysfunction in a mouse model of hemochromatosis.},
  author={Hani A Jouihan and Paul A Cobine and Robert C. Cooksey and Emily Hoagland and Sihem Boudina and E. Dale Abel and Dennis R Winge and Donald A. McClain},
  journal={Molecular medicine},
  year={2008},
  volume={14 3-4},
  pages={
          98-108
        }
}
Previous phenotyping of glucose homeostasis and insulin secretion in a mouse model of hereditary hemochromatosis (Hfe(-/-)) and iron overload suggested mitochondrial dysfunction. Mitochondria from Hfe(-/-) mouse liver exhibited decreased respiratory capacity and increased lipid peroxidation. Although the cytosol contained excess iron, Hfe(-/-) mitochondria contained normal iron but decreased copper, manganese, and zinc, associated with reduced activities of copper-dependent cytochrome c oxidase… CONTINUE READING
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