- Carnitine-acylcarnitine translocase
Name=solute carrier family 25 (carnitine/acylcarnitine translocase), member 20
Carnitine-acylcarnitine translocases are responsible for transporting both
carnitineand carnitine- fatty acidcomplexes into and out of the mitochondria, across the inner mitochondrial membrane.
This enzyme is required as fatty acids cannot cross the mitchondrial membranes without assistance. The fatty acid is firstly bound to
CoAto cross the external mitochondrial membrane. It then switches the CoAfor carnitineby 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 IIand 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 is 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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