Enzymology of the carnitine biosynthesis pathway

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


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.

Publication types

  • Review

MeSH terms

  • Aldehyde Oxidoreductases / metabolism
  • Aldehyde-Lyases / metabolism
  • Animals
  • Candida albicans / enzymology
  • Carnitine / biosynthesis*
  • Carnitine / deficiency
  • Carnitine / metabolism
  • Humans
  • Metabolic Networks and Pathways
  • Mixed Function Oxygenases / metabolism
  • gamma-Butyrobetaine Dioxygenase / metabolism


  • Mixed Function Oxygenases
  • gamma-Butyrobetaine Dioxygenase
  • epsilon-N-trimethyllysine hydroxylase
  • 4-N-trimethylaminobutyraldehyde dehydrogenase
  • Aldehyde Oxidoreductases
  • Aldehyde-Lyases
  • beta-hydroxy-trimethyllysine aldolase
  • Carnitine