Molecular enzymology of carnitine transfer and transport

Rona Ruth Ramsay, RD Gandour, F van der Leij

Research output: Contribution to journalArticlepeer-review

285 Citations (Scopus)

Abstract

Carnitine (L-3-hydroxy-4-N-trimethylaminobutyric acid) forms esters with a wide range of acyl groups and functions to transport and excrete these groups. It is found in most cells at millimolar levels after uptake via the sodium-dependent carrier, OCTN2. The acylation state of the mobile carnitine pool is linked to that of the limited and compartmentalised coenzyme A pools by the action of the family of carnitine acyltransferases and the mitochondrial membrane transporter, CACT. The genes and sequences of the carriers and the acyltransferases are reviewed along with mutations that affect activity. After summarising the accepted enzymatic background, recent molecular studies on the carnitine acyltransferases are described to provide a picture of the role and function of these freely reversible enzymes. The kinetic and chemical mechanisms are also discussed in relation to the different inhibitors under study for their potential to control diseases of lipid metabolism. (C) 2001 Elsevier Science B.V. All rights reserved.

Original languageEnglish
Pages (from-to)21-43
Number of pages23
JournalBiochimica et Biophysica Acta
Volume1546
DOIs
Publication statusPublished - 9 Mar 2001

Keywords

  • carnitine
  • OCTN2
  • CACT
  • carnitine acyltransferases
  • fatty acid oxidation
  • transport
  • molecular genetics
  • enzymology
  • inhibitor
  • FATTY-ACID OXIDATION
  • PALMITOYLTRANSFERASE-II DEFICIENCY
  • PROLIFERATOR-ACTIVATED RECEPTOR
  • MALONYL-COA SENSITIVITY
  • RAT-LIVER MITOCHONDRIA
  • ORGANIC CATION/CARNITINE TRANSPORTER
  • SITE-DIRECTED MUTAGENESIS
  • MESSENGER-RNA ABUNDANCE
  • BROWN ADIPOSE-TISSUE
  • N-TERMINAL DOMAIN

Fingerprint

Dive into the research topics of 'Molecular enzymology of carnitine transfer and transport'. Together they form a unique fingerprint.

Cite this