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IUBMB Life. 2010 May;62(5):357-62. doi: 10.1002/iub.323.

Enzymology of the carnitine biosynthesis pathway.

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1
Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.

Abstract

The water-soluble zwitterion carnitine is an essential metabolite in eukaryotes required for fatty acid oxidation as it functions as a carrier during transfer of activated acyl and acetyl groups across intracellular membranes. Most eukaryotes are able to synthesize carnitine endogenously, besides their capacity to take up carnitine from the diet or extracellular medium through plasma membrane transporters. This review discusses the current knowledge on carnitine homeostasis with special emphasis on the enzymology of the four steps of the carnitine biosynthesis pathway.

PMID:
20306513
DOI:
10.1002/iub.323
[Indexed for MEDLINE]
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