Mitochondrial carrier

Mitochondrial carriers are a family of transport proteins belonging to the carriers ( permeases ) and are found only in the organelle (mostly in mitochondria) membranes of eukaryotic cells. They consist of about 300 amino acids and have six α -helices in the secondary structure. An evolutionary standpoint, the basic structure is probably formed about two billion years ago by intra -genetic Gentriplikation a precursor with two α - helices.

The majority of these evolutionarily unrelated, mitochondrial carrier catalyzes antiport two substances such as keto acids, amino acids, nucleotides, inorganic ions, and cofactors.

The transport equation for the antiport is:

Outside of mitochondria mitochondrial carriers were found in peroxisomes of different animals, in hydrogenosomes anaerobic fungi living in plant and amyloplasts. The people are known about 50 of them; they are mainly encoded by the SLC25 genes. Mutations in eleven of these genes may be responsible for defects in each one of the proteins and be the cause of (rare ) genetic diseases, such as: defects in the ATP / ADP translocase, carnitine - acylcarnitine in the transporter, in the phosphate transporter and in the aspartate - glutamate carrier.