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Mitochondrial transporters are membrane-inserted proteins which provide a link between metabolic reactions occurring within the mitochondrial matrix and outside the organelles by catalyzing the translocation of numerous solutes across the mitochondrial membrane. They include the mitochondrial carrier family members, the proteins involved in pyruvate transport, ABC transporters and channels, and are, therefore, essential for many biological processes and cell homeostasis. Identification and functional studies of many mitochondrial transporters have been performed over the years using both in vitro and in vivo systems. The few recently solved structures of these transporters have paved the way for further investigations. Furthermore, alterations in their function are responsible for several diseases.

hypoxia --- resistance to hypoxia --- mitochondria --- mitochondrial calcium transport --- mitochondrial calcium uniporter complex --- mitochondrial Ca2+-induced permeability transition pore --- cyclophilin D --- ATP synthase --- disease --- error of metabolism --- mitochondrial carrier --- mitochondrial carrier disease --- mitochondrial disease --- mitochondrial transporter --- membrane transport --- mutation --- SLC25. --- mitochondrial permeability transition --- apoptosis --- necrosis --- ischemia/reperfusion --- cancer --- neurodegeneration --- cyclosporin A --- metabolite transport --- mitochondrial pyruvate carrier --- sideroflexin --- TOM --- TIM chaperones --- TIM22 --- protein translocation --- mitochondrial biogenesis --- amino acid --- biological function --- ion --- inner mitochondrial membrane --- mitochondrial carrier family --- organic acid --- substrate specificity --- transport mechanism --- vitamin --- USMG5/DAPIT --- glucose-stimulated insulin secretion --- glucose-induced expression --- membrane subunits of ATP synthase --- ATP synthase oligomers mitochondrial cristae morphology --- metabolism --- metabolic disorders --- adult-onset type II citrullinemia (CTLN2) --- aspartate/glutamate carrier (AGC) --- animal model --- argininosuccinate synthetase (ASS) --- aversion to carbohydrates --- citrin --- food taste --- neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) --- protein–protein interaction --- MPC --- lifespan --- pyruvate metabolism --- mitochondrial transport --- peroxisomes --- carrier --- cofactor --- ABC transporter --- aquaporin --- ion channels --- potassium channels --- ATP --- calcium --- ROS --- potassium channel openers --- MCF --- function --- plant metabolism --- plant development --- diseases --- VDAC1 --- virus --- pancreatic islets --- β-cell --- diabetes --- glucotoxicity --- glucolipotoxicity --- lipotoxicity --- mitochondrial carriers --- SLC transporters --- SLC25 --- SLC54 --- SLC55 --- LETM --- SLC56 --- sequence analysis --- protein targeting --- Voltage-Dependent Anion selective Channel --- isoforms --- oxidative post-translational modification --- gene promoter --- yeast --- bioenergetics --- SLC25A1 --- CIC --- CTP --- citrate --- inflammation --- 22.q11.2 --- NAFLD/NASH --- carnitine --- carnitine acyl-carnitine carrier --- carnitine acyl-carnitine translocase --- post-translational modification --- solute carrier family 25 --- SLC25A20 --- MCU --- mitochondrial Ca2+ uniporter --- Ca2+ signaling --- mitochondrial metabolism --- skeletal muscle mitochondria --- SLC25A51 --- NAD+ transporters --- NAD --- electrophysiology --- ATP-dependent potassium channel