Iron and copper in mitochondrial diseases.
review · Level V
Where this comes from
- Record sourced from PubMed, PMID 23473029.
- Also identified by DOI 10.1016/j.cmet.2013.02.004 and PMC identifier 3594794.
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Abstract
Transition metals are frequently used as cofactors for enzymes and oxygen-carrying proteins that take advantage of their propensity to gain and lose single electrons. Metals are particularly important in mitochondria, where they play essential roles in the production of ATP and detoxification of reactive oxygen species. At the same time, transition metals (particularly Fe and Cu) can promote the formation of harmful radicals, necessitating meticulous control of metal concentration and subcellular compartmentalization. We summarize our current understanding of Fe and Cu in mammalian mitochondrial biology and discuss human diseases associated with aberrations in mitochondrial metal homeostasis.
Medical subject headings
- Heme
- Homeostasis
- Iron
- Iron-Sulfur Proteins
- Mitochondrial Diseases
- Models, Biological