Phosphate carrier protein, mitochondrial (SLC25A3)

The phosphate carrier protein, mitochondrial (SLC25A3), is a multi-pass transmembrane transporter located in the inner mitochondrial membrane and belongs to the mitochondrial carrier family (SLC25)[1][5][7]. Its principal function is to import inorganic phosphate (Pi) into the mitochondrial matrix, which is essential for ATP production via oxidative phosphorylation[1][5][6]. SLC25A3 is also involved in mitochondrial copper transport, necessary for the activity of cytochrome c oxidase and thus for respiratory chain function[2][3][4]. There are two major isoforms, resulting from alternative splicing, with tissue-specific distribution (cardiac/skeletal muscle vs. ubiquitous)[2][3][5]. Mutations in SLC25A3 cause a severe, often fatal disorder characterized by lactic acidosis, hypotonia, cardiomyopathy, and early death[5][6]. Beyond energy metabolism, SLC25A3 regulates mitochondrial membrane potential, affects susceptibility to cell death via regulation (not direct formation) of the mitochondrial permeability transition pore, and plays a role in mitochondrial calcium homeostasis[1][6]. No approved drugs directly target SLC25A3, but it represents a potential therapeutic target for mitochondrial and metabolic diseases[1][6].

Other names

Solute carrier family 25 member 3SLC25A3Phosphate transport protein, mitochondrialPhosphate carrier (PHC)PiC2, PiCPTPOK/SW-cl.48

Not applicable for approved drugs, but research compounds indirectly modulate carrier activity, mitochondrial permeability, or metabolic flux through SLC25A3 in experimental models.