title: The elusive importance of being a mitochondrial Ca(2+) uniporter.
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.
authors.affiliation: Neuroscience Institute, National Research Council, Padova, Italy; Department of Biomedical Sciences, University of Padova, Italy.
authors.forename: Diana
authors.initials: D
authors.lastname: Pendin
authors.affiliation: Neuroscience Institute, National Research Council, Padova, Italy; Department of Biomedical Sciences, University of Padova, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Greotti
authors.affiliation: 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.
authors.forename: Tullio
authors.initials: T
authors.lastname: Pozzan
mesh_terms: D000818:Animals; D002118:Calcium / Q000378:metabolism; D015220:Calcium Channels / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D033681:Mitochondrial Membrane Transport Proteins / Q000737:chemistry / Q000378:metabolism; D021122:Protein Subunits / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D034622:RNA Interference
chemical_list: D015220:Calcium Channels; D033681:Mitochondrial Membrane Transport Proteins; D021122:Protein Subunits; C585180:SMDT1 protein, human; C034703:mitochondrial calcium uniporter; D002118:Calcium
keywords: EMRE; MCU; MCUR; MICU1; MICU2; Mitochondrial Ca(2+) uniporter