Fatty acid oxidation occurs in the mitochondrial matrix. However, long-chain fatty acyl-CoA molecules cannot cross the inner membrane to enter the matrix. The carnitine shuttle system transfers the acyl group from CoA to carnitine, which can enter the mitochondrial matrix. Label the enzymes and compounds of the carnitine shuttle system. These abbreviations are used: intermembrane space, IMS; carnitine acyltransferase I, CAT1; mitochondrial carnitine acyltransferase II, CAT2; and carnitine/acylcarnitine translocase (carnitine carrier protein), CAT.

The carnitine shuttle system is an important system that helps in the fatty acid oxidation.

The functions of the enzymes and compounds of the carnitine shuttle system include the following:

Intermembrane space (IMS): The activities of the enzymes of the carnitine system takes place in this space within the cells.Carnitine acyltransferase I, (CAT1): This is also called Carnitine palmitoyltransferase 1. It converts long-chain acyl-CoA species to their corresponding long-chain acyl-carnitines for transport into the mitochondria.Mitochondrial Carnitine acyltransferase II, CAT2: This is an enzyme found inside the mitochondria that oxidizes long-chain fatty acids in the mitochondria.carnitine/acylcarnitine translocase (carnitine carrier protein), CAT: This is a membrane transporter that exchanges cytoplasmic acylcarnitine for mitochondrial carnitine.

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