Carnitine, mitochondrial function and therapy

Victor A. Zammit, Rona R. Ramsay, Mario Bonomini, Arduino Arduini

Research output: Contribution to journalReview articlepeer-review

114 Citations (Scopus)

Abstract

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. (C) 2009 Elsevier B.V. All rights reserved.

Original languageEnglish
Pages (from-to)1353-1362
Number of pages10
JournalAdvanced Drug Delivery Reviews
Volume61
Issue number14
DOIs
Publication statusPublished - 30 Nov 2009

Keywords

  • Carnitine
  • Mitochondria
  • Coenzyme A
  • Carnitine acyltransferases
  • PALMITOYLTRANSFERASE-I DEFICIENCY
  • PYRUVATE-DEHYDROGENASE COMPLEX
  • HUMAN SKELETAL-MUSCLE
  • FATTY-ACID OXIDATION
  • MAINTENANCE HEMODIALYSIS-PATIENTS
  • TYPE-2 DIABETES-MELLITUS
  • RAT-LIVER MITOCHONDRIA
  • COENZYME-A METABOLISM
  • N-TERMINAL DOMAIN
  • CHAIN ACYL-COA

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