Carnitine biosynthesis in mammals.
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.
Vaz
Frédéric M
FM
Laboratory for Genetic Metabolic Diseases, Departments of Clinical Chemistry and Paediatrics, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands. f.m.vaz@amc.uva.nl
Wanders
Ronald J A
RJ
D000595:Amino Acid Sequence; D000818:Animals; D002331:Carnitine / Q000096:biosynthesis*; D006801:Humans; D006901:Hydroxylysine / Q000031:analogs & derivatives* / Q000378:metabolism; D008099:Liver / Q000378:metabolism; D006899:Mixed Function Oxygenases / Q000378:metabolism; D008954:Models, Biological; D008956:Models, Chemical; D008969:Molecular Sequence Data; D010100:Oxygen / Q000378:metabolism; D051381:Rats; D017386:Sequence Homology, Amino Acid; D005680:gamma-Aminobutyric Acid / Q000031:analogs & derivatives / Q000378:metabolism
C025930:3-hydroxy-N(6)-trimethyl-lysine; D006901:Hydroxylysine; D005680:gamma-Aminobutyric Acid; C029081:trimethylaminobutyrate; D006899:Mixed Function Oxygenases; C025931:trimethyl-lysine hydroxylase; D002331:Carnitine; D010100:Oxygen