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Carnitine Biosynthesis Nutritional Implications

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Advances in Nutritional Research

Part of the book series: Advances in Nutritional Research ((ANUR,volume 4))

Abstract

Much interest in carnitine metabolism and function has been shown in recent years with the recognition of its catalytic role in the intramitochondrial transport of fatty acids. Figure 1 shows a series of events requisite for the activation and transport of extramitochondrial long-chain fatty acids such as palmitic acid to the site of β-oxidation in the mitochondrial matrix [cf. Bremer (1977) for discussion and relevant references]. Palmitic acid released from adipose tissue or derived from the diet is activated by outer membrane ATP-dependent palmitoyl-CoA synthetase to form palmitoyl-CoA. Such long-chain fatty acyl-CoA esters have only a limited ability to cross the mitochondrial membrane, but their entry is facilitated by “outer” carnitine palmitoyl transferase which catalyzes a transes-terification reaction in which the palmitoyl moiety from CoA is transferred to carnitine, forming palmitoylcarnitine. The latter ester is then thought to cross the inner mitochondrial membrane through the action of a translocase. A second transesterification reaction now takes place wherein “inner” palmitoyltransferase, located in the inner mitochondrial membrane, regenerates palmitoyl-CoA for subsequent β-oxidation and releases carnitine for a repetition of its catalytic role in overall fatty acid transport.

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Authors and Affiliations

  1. Division of Nutrition, Department of Biochemistry, Vanderbilt University, Nashville, Tennessee, 37232, USA

    Harry P. Broquist & Peggy R. Borum

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  1. Harry P. Broquist
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  1. University of Guelph, Guelph, Ontario, Canada

    Harold H. Draper

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Broquist, H.P., Borum, P.R. (1982). Carnitine Biosynthesis Nutritional Implications. In: Draper, H.H. (eds) Advances in Nutritional Research. Advances in Nutritional Research, vol 4. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-9934-6_7

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