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PMID: 11802770 Published · ppublish English Journal Article Review

Carnitine biosynthesis in mammals.

The Biochemical journal ·Vol. 361 ·No. Pt 3 ·2002-02-01 ·Pages 417-29

Vaz FM, Wanders RJ

Abstract

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.

MeSH Terms
Amino Acid Sequence Animals Carnitine/biosynthesis Humans Hydroxylysine/analogs & derivatives,metabolism Liver/metabolism Mixed Function Oxygenases/metabolism Models, Biological Models, Chemical Molecular Sequence Data Oxygen/metabolism Rats Sequence Homology, Amino Acid gamma-Aminobutyric Acid/analogs & derivatives,metabolism
Chemicals
3-hydroxy-N(6)-trimethyl-lysine Hydroxylysine gamma-Aminobutyric Acid trimethylaminobutyrate Mixed Function Oxygenases trimethyl-lysine hydroxylase Carnitine Oxygen
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Vaz Frédéric M
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. [email protected]
Wanders Ronald J A
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Article Info
Journal
The Biochemical journal
Abbr.
Biochem J
ISSN
0264-6021
DOI
10.1042/0264-6021:3610417
Email
[email protected]
Published
2002-02-01
Pages
417-29
Language
English
Region
England
NLM ID
2984726R
PMCID
PMC1222323
Subset
IM
External Links
PubMed source DOI: 10.1042/0264-6021:3610417 PMC Full Text View journal details
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