title: The mitochondrial phosphate carrier: Role in oxidative metabolism, calcium handling and mitochondrial disease.
abstract: The mitochondrial phosphate carrier (PiC) is a mitochondrial solute carrier protein, which is encoded by SLC25A3 in humans. PiC delivers phosphate, a key substrate of oxidative phosphorylation, across the inner mitochondrial membrane. This transport activity is also relevant for allowing effective mitochondrial calcium handling. Furthermore, PiC has also been described to affect cell survival mechanisms via interactions with cyclophilin D and the viral mitochondrial-localized inhibitor of apoptosis (vMIA). The significance of PiC has been supported by the recent discovery of a fatal human condition associated with PiC mutations. Here, we present first the early studies that lead to the discovery and molecular characterization of the PiC, then discuss the very recently developed mouse models for PiC and pathological mutations in the human SLC25A3 gene.
authors.affiliation: MitoCare Center, Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA. Electronic address: erin.seifert@jefferson.edu.
authors.forename: Erin L
authors.initials: EL
authors.lastname: Seifert
authors.affiliation: Department of Physiology, Semmelweis University, Budapest 1085, Hungary.
authors.forename: Erzsébet
authors.initials: E
authors.lastname: Ligeti
authors.affiliation: Department of Paediatrics, Paracelsus Medical University, SALK Salzburg, Salzburg 5020, Austria.
authors.forename: Johannes A
authors.initials: JA
authors.lastname: Mayr
authors.affiliation: Department of Pediatrics, University of Pennsylvania, Philadelphia, PA 19104, USA.
authors.forename: Neal
authors.initials: N
authors.lastname: Sondheimer
authors.affiliation: MitoCare Center, Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA. Electronic address: gyorgy.hajnoczky@jefferson.edu.
authors.forename: György
authors.initials: G
authors.lastname: Hajnóczky
mesh_terms: D000818:Animals; D002118:Calcium / Q000378:metabolism*; D003001:Cloning, Molecular; D006801:Humans; D051379:Mice; D028361:Mitochondrial Diseases / Q000378:metabolism / Q000503:physiopathology*; D033681:Mitochondrial Membrane Transport Proteins / Q000235:genetics / Q000378:metabolism / Q000502:physiology*; D009154:Mutation; D010084:Oxidation-Reduction; D010710:Phosphates / Q000378:metabolism*
chemical_list: D033681:Mitochondrial Membrane Transport Proteins; D010710:Phosphates; D002118:Calcium