Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2014 Mar 1;592(5):829-39.
doi: 10.1113/jphysiol.2013.268235. Epub 2013 Dec 23.

The mitochondrial calcium uniporter complex: molecular components, structure and physiopathological implications

Saverio Marchi  1 , Paolo Pinton
Affiliations
Review

The mitochondrial calcium uniporter complex: molecular components, structure and physiopathological implications

Saverio Marchi et al. J Physiol. .

Abstract

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.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Ion fluxes are indicated by arrows. The lower magnification represents the different components of the uniporter complex. MCU Oligomerization at IMM forms a tetramer (shadowed subunits). MCUb is represented as the black shadowed subunit. SERCA, sarcoendoplasmic reticulum calcium transport ATPase; VDAC, voltage-dependent anion channel; TMD, transmembrane domain; RuR, Ruthenium Red. See text for further details.
Figure 2
Figure 2
Dynamic diagram showing Ca2+ changes in the different states of cytosolic Ca2+ upon MICU1 knock-down
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Alam MR, Groschner LN, Parichatikanond W, Kuo L, Bondarenko AI, Rost R, Waldeck-Weiermair M, Malli R, Graier WF. Mitochondrial Ca2+ uptake 1 (MICU1) and mitochondrial Ca2+ uniporter (MCU) contribute to metabolism-secretion coupling in clonal pancreatic beta-cells. J Biol Chem. 2012;287:34445–34454. - PMC - PubMed
    1. Altschuld RA, Hohl CM, Castillo LC, Garleb AA, Starling RC, Brierley GP. Cyclosporin inhibits mitochondrial calcium efflux in isolated adult rat ventricular cardiomyocytes. Am J Physiol Heart Circ Physiol. 1992;262:H1699–H1704. - PubMed
    1. Baughman JM, Perocchi F, Girgis HS, Plovanich M, Belcher-Timme CA, Sancak Y, Bao XR, Strittmatter L, Goldberger O, Bogorad RL, Koteliansky V, Mootha VK. Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter. Nature. 2011;476:341–345. - PMC - PubMed
    1. Bick AG, Calvo SE, Mootha VK. Evolutionary diversity of the mitochondrial calcium uniporter. Science. 2012;336:886. - PMC - PubMed
    1. Bonora M, Giorgi C, Bononi A, Marchi S, Patergnani S, Rimessi A, Rizzuto R, Pinton P. Subcellular calcium measurements in mammalian cells using jellyfish photoprotein aequorin-based probes. Nat Protoc. 2013;8:2105–2118. - PubMed

Publication types

MeSH terms