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Mitochondrial CPT System

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Carnitine (L-Carnitine or 3-hydroxy-4-(trimethylammonio) butanoate) and its Acyl-Esters (Acyl-Carnitines or ROC-Carnitine) are essential compounds for the metabolism of fatty acids (or RCOOH compounds). Carnitine is synthesized de novo in animal cells, but it is believed that most comes from the diet. Its main function is to assist the transport and metabolism of fatty acids into mitochondria, where they are oxidized for energy production. Carnitine maintains a balance between free and esterified CoA (Coenzyme A or CoASH), since an excess of Acyl-CoA (or ROC-CoA) intermediates are potentially toxic to cells. In addition, Carnitine is required to remove any surplus of Acyl groups from mitochondria. Fatty acids are first activated by CoA, i.e. to form highly polar Thiol esters, Acyl-CoA, on the [...]


1.Regulation of liver carnitine palmitoyltransferase I gene expression by hormones and fatty acids.
Louet JF, Le May C, Pegorier JP, Decaux JF, Girard J.
Biochem. Soc. Trans. 2001 May;29(Pt 2):310-6.
2.Molecular characterization of human acetyl-CoA synthetase, an enzyme regulated by sterol regulatory element-binding proteins.
Luong A, Hannah VC, Brown MS, Goldstein JL.
J. Biol. Chem. 2000 Aug 25;275(34):26458-66.
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