ATP–ADP translocase

ATP / ADP translocase (AAC ), the protein in the mitochondrial membrane of eukaryotes, which catalyzes the transport of ATP in the mitochondria; simultaneously ADP is funneled into the cytosol. Transportation is an essential component of energy metabolism, as ATP is required throughout the cell as an energy supplier.

In man, there are four paralogous isoforms of handling, which are located in different tissue types. Mutations in the SLC25A4 gene, which encodes AAC1, are responsible for a rare genetic disease that is associated with ophthalmoplegia externa progressiva (paralysis of the eye muscles ).

The transport equation is:

It is an antiporter, and he is thereby operated to ADP or ATP are transported without binding magnesium ions. ATP is thus fourfold negatively charged at pH 7, ADP triple negative. ATP thus travels preferentially along the membrane potential from the matrix in the IMR.

AAC is a common protein of the inner membrane of the mitochondrion and can constitute up to 14% of all proteins of the inner membrane. Consequently, it is unlikely that the ATP synthesis due to lack of ADP is limited. The translocase by atractyloside can, a glycoside, are inhibited. Also bongkrekic acid inhibits the translocator. This binds to the matrix side of the protein and consequently must only enter the Mitochondriummatrix. Bongkrekic acid is excreted from the bacterium Pseudomonas cocovenenas.