Mitochondrial metals as a potential therapeutic target in neurodegeneration

A. Grubman, A R White, J. R. Liddell

Research output: Contribution to journalReview ArticleResearchpeer-review

25 Citations (Scopus)


Transition metals are critical for enzyme function and protein folding, but in excess can mediate neurotoxic oxidative processes. As mitochondria are particularly vulnerable to oxidative damage due to radicals generated during ATP production, mitochondrial biometal homeostasis must therefore be tightly controlled to safely harness the redox potential of metal enzyme cofactors. Dysregulation of metal functions is evident in numerous neurological disorders including Alzheimer's disease, stroke, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis and Friedrich's ataxia. This review describes the mitochondrial metal defects in these disorders and highlights novel metal-based therapeutic approaches that target mitochondrial metal homeostasis in neurological disorders.
Original languageEnglish
Pages (from-to)2159-2173
Number of pages15
JournalBritish Journal of Pharmacology
Issue number8
Publication statusPublished - Apr 2014
Externally publishedYes


  • biometal homeostasis
  • Cu(atsm)
  • curcumin
  • deferiprone
  • mitochondria
  • Mn porphyrins
  • neurodegeneration
  • stroke

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