Carnitine, mitochondrial function and therapy

Adv Drug Deliv Rev. 2009 Nov 30;61(14):1353-62. doi: 10.1016/j.addr.2009.04.024. Epub 2009 Aug 26.


Carnitine is important for cell function and survival primarily because of its involvement in the multiple equilibria between acylcarnitine and acyl-CoA esters established through the enzymatic activities of the family of carnitine acyltransferases. These have different acyl chain-length specificities and intracellular compartment distributions, and act in synchrony to regulate multiple aspects of metabolism, ranging from fuel-selection and -sensing, to the modulation of the signal transduction mechanisms involved in many homeostatic systems. This review aims to rationalise the extensive range of experimental and clinical data that have been obtained through the pharmacological use of L-carnitine and its short-chain acylesters, over the past two decades, in terms of the basic biochemical mechanisms involved in the effects of carnitine on the various cellular acyl-CoA pools in health and disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Carnitine / analogs & derivatives
  • Carnitine / metabolism*
  • Carnitine / therapeutic use*
  • Carnitine Acyltransferases / metabolism
  • Heart Diseases / drug therapy*
  • Humans
  • Mitochondria / drug effects
  • Mitochondria / metabolism*


  • acylcarnitine
  • Carnitine Acyltransferases
  • Carnitine