Carnitine-acylcarnitine translocase Totally Explained

Everything about Carnitine-acylcarnitine Translocase totally explained

Carnitine-acylcarnitine translocases are responsible for transporting both carnitine and carnitine-fattyacid complexes into and out of the mitochondria, across the inner mitochondrial membrane. This enzyme is required as fatty acids can't cross the mitchondrial membranes without assistance. The fatty acid is firstly bound to CoA to cross the external mitochondrial membrane. It then switches the CoA for carnitine by the use of the enzyme carnitine palmitoyl transferase I. The complex then uses facilitated diffusion by Carnitine-acylcarnitine translocase to enter the mitochondrial matrix. Here, the acylcartinine complex is disrupted by cartinine palmitoyl transferase II and the fatty acid rebinds to CoA. Cartinine then diffuses back across the membrane by a second carnitine-acylcarnitine translocase into the inter-mitochondiral membrane space. This is the cartinine shuttle system.
A disorder is associated with carnitine-acylcarnitine translocase deficiency. This disorder prevents the shuttle-like action of cartinine from assisting fatty acids across the mitochondiral membrane and therefore there's decreased fatty acid metabolism. The result of this is an increased number of fat droplets within muscles and liver, decreased tolerance to long term excerise, inability to fast for more than a few hours, muscle weakness and wasting, strong acidic smell on breath (due to protein breakdown)

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