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Biochem Biophys Res Commun. 2015 Aug 21;464(2):369-75. doi: 10.1016/j.bbrc.2015.06.031. Epub 2015 Jun 16.

The mitochondrial phosphate carrier: Role in oxidative metabolism, calcium handling and mitochondrial disease.

Author information

1
MitoCare Center, Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA. Electronic address: erin.seifert@jefferson.edu.
2
Department of Physiology, Semmelweis University, Budapest 1085, Hungary.
3
Department of Paediatrics, Paracelsus Medical University, SALK Salzburg, Salzburg 5020, Austria.
4
Department of Pediatrics, University of Pennsylvania, Philadelphia, PA 19104, USA.
5
MitoCare Center, Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA. Electronic address: gyorgy.hajnoczky@jefferson.edu.

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.

PMID:
26091567
DOI:
10.1016/j.bbrc.2015.06.031
[Indexed for MEDLINE]
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