Iron and copper in mitochondrial diseases.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Xu, W; Barrientos, T; Andrews, NC

Published Date

  • March 5, 2013

Published In

Volume / Issue

  • 17 / 3

Start / End Page

  • 319 - 328

PubMed ID

  • 23473029

Pubmed Central ID

  • PMC3594794

Electronic International Standard Serial Number (EISSN)

  • 1932-7420

Digital Object Identifier (DOI)

  • 10.1016/j.cmet.2013.02.004


  • eng

Conference Location

  • United States