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Defects of mitochondrial carriers that supply mitochondria with the substrates of oxidative phosphorylation, inorganic phosphate and ADP, are responsible for diseases characterized by defective energy production. [15] Residues involved in substrate binding in the middle of the transporter and gating have been identified and analyzed. [8]
Depiction of mitochondrial membranes. [1] Mitochondrial membrane transport proteins, also known as mitochondrial carrier proteins, are proteins which exist in the membranes of mitochondria. They serve to transport [2] molecules and other factors, such as ions, into or out of the organelles. Mitochondria contain both an inner and outer membrane ...
The structure of the tricarboxylate transport protein is consistent with the structures of other mitochondrial carriers. [9] [10] [12] In particular, the tricarboxylate transport protein has a tripartite structure consisting of three repeated domains that are approximately 100 amino acids in length.
The mitochondrial pyruvate carriers are composed of: [1] [2] Mitochondrial pyruvate carrier 1; Mitochondrial pyruvate carrier 2; The pyruvate carriers are involved in mitochondrial metabolism but it is possible to compensate for their loss of function. [3] They have been studied for a role in cardiac stress adaption. [4]
Phosphate carrier protein, mitochondrial is a protein that in humans is encoded by the SLC25A3 gene. [5] [6] The encoded protein is a transmembrane protein located in the mitochondrial inner membrane and catalyzes the transport of phosphate ions across it for the purpose of oxidative phosphorylation.
Mitochondrial pyruvate carrier 1 (MPC1), also known as brain protein 44-like (BRP44L) and SLC54A1, is a protein that in humans is encoded by the MPC1 gene. [5] It is part of the Mitochondrial Pyruvate Carrier (MPC) protein family .
The mitochondrial dicarboxylate carrier (DIC) is an integral membrane protein encoded by the SLC25A10 gene in humans that catalyzes the transport of dicarboxylates such as malonate, malate, and succinate across the inner mitochondrial membrane in exchange for phosphate, sulfate, and thiosulfate by a simultaneous antiport mechanism, thus supplying substrates for the Krebs cycle, gluconeogenesis ...
Mitochondrial 2-oxodicarboxylate carrier also known as solute carrier family 25 member 21 (SLC25A21) is a protein that in humans is encoded by the SLC25A21 gene. [5]It is a homolog of the S. cerevisiae ODC proteins, mitochondrial carriers that transport C5-C7 oxodicarboxylates across inner mitochondrial membranes.