Journal
BIOCHEMICAL JOURNAL
Volume 361, Issue -, Pages 417-429Publisher
PORTLAND PRESS LTD
DOI: 10.1042/0264-6021:3610417
Keywords
butyrobetaine; fatty acid metabolism; hydroxytrimethyl-lysine; trimethyl-lysine
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Carnitine is indispensable for energy metabolism, since it enables activated fatty acids to enter the mitochondria, where they are broken down via beta-oxidation. Carnitine is probably present in all animal species, and in numerous micro-organisms and plants. In mammals, carnitine homoeostasis is maintained by endogenous synthesis, absorption from dietary sources and efficient tubular reabsorption by the kidney. This review aims to cover the current knowledge of the enzymological, molecular, metabolic and regulatory aspects of mammalian carnitine biosynthesis, with an emphasis on the human and rat.
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