Fatty acids must be activated before they can be carried into the mitochondria, where fatty acid oxidation occurs. This process occurs in two steps catalyzed by the enzyme fatty acyl-CoA synthetase.
Transport into the mitochondrial matrix
Once activated, the acyl CoA is transported into the mitochondrial matrix. This occurs via a series of similar steps:
Acyl CoA is conjugated to carnitine by carnitine acyltransferase (palmitoyltransferase) I located on the outer mitochondrial membrane
Acyl carnitine is shuttled inside by a translocase
Acyl carnitine is converted to acyl CoA by carnitine acyltransferase (palmitoyltransferase) II located on the inner mitochondrial membrane. The liberated carnitine returns to the cytosol.
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