L-Carnitine Fumarate

Role in fatty acid metabolism Carnitine transports long-chain acyl groups from fatty acids into the , so that they can be broken down through to to obtain usable energy via the . In some organisms such as fungi, the acetate is used in the for and formation of . Fatty acids must be activated before binding to the carnitine molecule to form acyl-carnitine. The free fatty acid in the cytosol is attached with a bond to (CoA). This reaction is catalyzed by the enzyme and driven to completion by . The acyl group on CoA can now be transferred to carnitine and the resulting acyl-carnitine transported into the mitochondrial . This occurs via a series of similar steps: Acyl-CoA is conjugated to carnitine by (palmitoyltransferase) located on the outer mitochondrial membrane Acyl-carnitine is shuttled inside by a Acyl-carnitine is converted to acyl-CoA by (palmitoyltransferase) located on the inner mitochondrial membrane. The liberated carnitine returns to the cytosol. Human genetic disorders such as , , and affect different steps of this process. Carnitine acyltransferase I undergoes inhibition as a result of , an intermediate in fatty acid biosynthesis, in order to prevent futile cycling between β-oxidation and fatty acid synthesis.