Carnitine transport system

The carnitine transport system consists of two transport proteins – carnitine acyltransferase-1 and 2 (CAT-1,2) – on the outer and inner sides of the mitochondrial membrane. It serves to transfer fatty acids from the cytosol to the mitochondrial matrix. Fatty acids are activated in the cytosol to acyl-CoA by acyl-CoA synthetase . However, coenzyme A is too large a molecule and would not pass through the membrane. Thus, through CAT-1, the acyl is transferred to carnitine. The resulting acylcarnitine diffuses through the intermembrane space to the CAT-2 transporter, where the acyl is transferred across the membrane and reactivated to acyl-CoA.

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