Mitochondrial 2-oxodicarboxylate carrier (SLC25A21)

SLC25A21 (Mitochondrial 2-oxodicarboxylate carrier) is a transporter protein located on the inner mitochondrial membrane that mediates the exchange of C5-C7 oxodicarboxylates, notably importing 2-oxoadipate and exporting 2-oxoglutarate[1][2][3][5]. It plays a critical role in the catabolism of lysine, hydroxylysine, and tryptophan, importing 2-oxoadipate—a key intermediate—into the mitochondria where it is converted to acetyl-CoA and funneled into the citric acid cycle[1][2][3]. Pathogenic variants can cause mitochondrial DNA depletion syndrome 18 and neuromuscular disease by disrupting amino acid breakdown and mitochondrial energy metabolism[3][1]. SLC25A21 is also being investigated as a prognostic marker in cancers such as acute myeloid leukemia, reflecting its broader importance in mitochondrial function and apoptosis regulation[4].

Other names

Mitochondrial 2-oxoadipate carrierSolute carrier family 25 member 21ODCODC1MTDPS18oxodicarboxylate carriersolute carrier family 25 (mitochondrial oxoadipate carrier) member 21solute carrier family 25 (mitochondrial oxodicarboxylate carrier) member 21