Tricarboxylate transport protein, mitochondrial (SLC25A1)

Tricarboxylate transport protein, mitochondrial (SLC25A1) is a mitochondrial inner membrane transporter protein that exchanges citrate (from the mitochondrial matrix) for malate (from the cytosol)[1][3]. This carrier plays a central role in cellular metabolism by exporting mitochondrial citrate, a precursor for fatty acid and cholesterol biosynthesis, and regulating key metabolic processes including glycolysis and energy production[1][3]. SLC25A1 is essential for maintaining cytosolic acetyl-CoA levels and NADPH generation via the citrate–malate shuttle. Mutations in SLC25A1 disrupt this exchange, leading to combined D,L-2-hydroxyglutaric aciduria, an inborn error of metabolism with prominent neurological symptoms resulting from abnormal organic acid accumulation and impaired energy metabolism[1][3]. SLC25A1 is part of the larger mitochondrial carrier family (SLC25), whose members share structural features and a common transport mechanism across the mitochondrial inner membrane[2]. Altered SLC25A1 activity or expression has also been implicated in cancer metabolic reprogramming and possibly other diseases[3].

Other names

SLC25A1Tricarboxylate carrier proteinCitrate transport proteinCICCTPCMS23D2L2ADSEASLC20A3Mitochondrial citrate carrierCitrate–isocitrate carrierSolute carrier family 25 member 1

Inhibition of mitochondrial citrate export; disruption of cellular acetyl-CoA and lipid synthesis; modulation of metabolic flux between mitochondria and cytosol