Molecular enzymology of carnitine transfer and transport

Biochim Biophys Acta. 2001 Mar 9;1546(1):21-43. doi: 10.1016/s0167-4838(01)00147-9.


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.

Publication types

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

MeSH terms

  • Acyl Coenzyme A / metabolism*
  • Amino Acid Sequence
  • Animals
  • Biological Transport
  • Carnitine / analogs & derivatives
  • Carnitine / metabolism*
  • Carnitine Acyltransferases / chemistry
  • Carnitine Acyltransferases / genetics
  • Carnitine O-Palmitoyltransferase / chemistry
  • Carnitine O-Palmitoyltransferase / genetics
  • Carrier Proteins / chemistry
  • Carrier Proteins / genetics
  • Carrier Proteins / metabolism*
  • Cell Membrane / metabolism
  • Endoplasmic Reticulum / metabolism
  • Humans
  • Intracellular Membranes / metabolism
  • Liver / metabolism
  • Membrane Proteins / chemistry
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism*
  • Models, Chemical
  • Models, Molecular
  • Molecular Sequence Data
  • Molecular Structure
  • Organic Cation Transport Proteins*
  • Plants
  • Sequence Alignment
  • Solute Carrier Family 22 Member 5


  • Acyl Coenzyme A
  • Carrier Proteins
  • Membrane Proteins
  • Organic Cation Transport Proteins
  • SLC22A5 protein, human
  • Solute Carrier Family 22 Member 5
  • Carnitine Acyltransferases
  • Carnitine O-Palmitoyltransferase
  • Carnitine