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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 ...
Mitoferrin-1 (Mfrn1) is a 38 kDa protein [5] that is encoded by the SLC25A37 gene in humans. [6] [7] It is a member of the Mitochondrial carrier (MC) Superfamily, however, its metal cargo makes it distinct from other members of this family.
MTCH2 assists in the recruitment of BID into the mitochondria during apoptosis. [9] Variants of the MTCH2 gene may be associated with obesity. [10] MTCH2 represses mitochondrial metabolism such that a deficiency of MTCH2 increases energy consumption and production by mitochondria. [9]
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.
Adenine nucleotide translocator (ANT), also known as the ADP/ATP translocase (ANT), ADP/ATP carrier protein (AAC) or mitochondrial ADP/ATP carrier, exchanges free ATP with free ADP across the inner mitochondrial membrane. [1] [2] ANT is the most abundant protein in the inner mitochondrial membrane and belongs to the mitochondrial carrier family ...
The mobile cytochrome electron carrier in mitochondria is cytochrome c. Bacteria use a number of different mobile cytochrome electron carriers. Other cytochromes are found within macromolecules such as Complex III and Complex IV. They also function as electron carriers, but in a very different, intramolecular, solid-state environment.
They also reduce the mitochondrial membrane potential in mammalian cells, which reduces production of reactive oxygen species (ROS). In contrast to UCP1 and UCP3, which are primarily expressed in adipose and smooth muscle, UCP2 is expressed on many different tissues [ 6 ] including the kidney, liver, GI tract, brain, and skeletal muscle.