The role of the carnitine system in peroxisomal fatty acid oxidation

Rona Ruth Ramsay

Research output: Contribution to journalArticlepeer-review

47 Citations (Scopus)

Abstract

Peroxisomes are small, subcellular organelles that play a major role in lipid metabolism. Inherited disorders of peroxisomal structure and metabolism can result from defective assembly, missing protein import transporters, or individual enzyme deficiencies. Molecular studies helped by the range of disorders have now elucidated many of the pathways, including the paths of a-oxidation for phytanic acid and P-oxidation for very-long-chain and branched-chain fatty acids and for bile acid synthesis. The mechanism of the transfer of substrates, intermediates, and products across the membrane is poorly understood. The carnitine system, known to transport activated acyl groups between localized coenzyme A pools, is presented. The evidence for the involvement of carnitine in the transfer of activated acyl groups to and from the peroxisomes is reviewed.

Original languageEnglish
Pages (from-to)28-35
Number of pages8
JournalAmerican Journal of the Medical Sciences
Volume318
Issue number1
Publication statusPublished - Jul 1999

Keywords

  • peroxisomes
  • beta-oxidation
  • VLCFA (very-long-chain fatty acids)
  • carnitine
  • carnitine octanoyltransferases
  • RAT-LIVER PEROXISOMES
  • BETA-OXIDATION
  • SACCHAROMYCES-CEREVISIAE
  • ACETYL-COA
  • ADRENOLEUKODYSTROPHY
  • PROTEIN
  • GENE
  • ACYLTRANSFERASES
  • MITOCHONDRIAL
  • MEMBRANE

Fingerprint

Dive into the research topics of 'The role of the carnitine system in peroxisomal fatty acid oxidation'. Together they form a unique fingerprint.

Cite this