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Cell Calcium. 2014 Mar;55(3):139-45. doi: 10.1016/j.ceca.2014.02.008. Epub 2014 Feb 17.

The elusive importance of being a mitochondrial Ca(2+) uniporter.

Author information

1
Neuroscience Institute, National Research Council, Padova, Italy; Department of Biomedical Sciences, University of Padova, Italy.
2
Neuroscience Institute, National Research Council, Padova, Italy; Department of Biomedical Sciences, University of Padova, Italy; Venetian Institute of Molecular Medicine, Padova, Italy. Electronic address: tullio.pozzan@unipd.it.

Abstract

The molecular components of the mitochondrial Ca(2+) uptake machinery have been only recently identified. In the last months, in addition to the pore forming subunit and of one regulatory protein (named MCU and MICU1, respectively) other four components of this complex have been described. In addition, a MCU KO mouse model has been generated and a genetic human disease due to missense mutation of MICU1 has been discovered. In this contribution, we will first summarize the recent findings, discussing the roles of the different subunits of the mitochondrial Ca(2+) uptake complex, pointing to the current contradictions in the published data, as well as possible explanations. Finally we will speculate on the recent, totally unexpected, results obtained in the MCU knock-out (KO) mice.

KEYWORDS:

EMRE; MCU; MCUR; MICU1; MICU2; Mitochondrial Ca(2+) uniporter

PMID:
24631327
DOI:
10.1016/j.ceca.2014.02.008
[Indexed for MEDLINE]
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