The Mitochondrial Calcium Uniporter Complex: Molecular Components, Structure and Physiopathological Implications

J Physiol. 2014 Mar 1;592(5):829-39. doi: 10.1113/jphysiol.2013.268235. Epub 2013 Dec 23.


Although it has long been known that mitochondria take up Ca2+, the molecular identities of the channels and transporters involved in this process were revealed only recently. Here, we discuss the recent work that has led to the characterization of the mitochondrial calcium uniporter complex, which includes the channel-forming subunit MCU (mitochondrial calcium uniporter) and its regulators MICU1, MICU2, MCUb, EMRE, MCUR1 and miR-25. We review not only the biochemical identities and structures of the proteins required for mitochondrial Ca2+ uptake but also their implications in different physiopathological contexts.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Calcium / metabolism*
  • Calcium Channels / chemistry*
  • Calcium Channels / metabolism*
  • Calcium Channels / ultrastructure
  • Calcium Signaling / physiology*
  • Humans
  • Membrane Potential, Mitochondrial / physiology*
  • Mitochondria / chemistry
  • Mitochondria / physiology*
  • Mitochondria / ultrastructure
  • Mitochondrial Diseases / metabolism*
  • Mitochondrial Diseases / pathology
  • Models, Biological
  • Models, Chemical
  • Structure-Activity Relationship


  • Calcium Channels
  • mitochondrial calcium uniporter
  • Calcium