Peroxisomes, lipid metabolism, and peroxisomal disorders

Mol Genet Metab. Sep-Oct 2004;83(1-2):16-27. doi: 10.1016/j.ymgme.2004.08.016.


Peroxisomes catalyse a large variety of different cellular functions of which most have to do with lipid metabolism. This paper deals with the role of peroxisomes in three key pathways of lipid metabolism, including: (1) etherphospholipid biosynthesis, (2) fatty acid beta-oxidation, and (3) fatty acid alpha-oxidation. Apart from a brief description of the peroxisomal enzymes involved in each of these pathways, the interaction between peroxisomes and other subcellular organelles, notably microsomes and peroxisomes, will be discussed. Finally, the current state of knowledge with respect to the different disorders of peroxisomal lipid metabolism will be described.

Publication types

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

MeSH terms

  • Acyl-CoA Oxidase / deficiency
  • Biological Transport
  • Fatty Acids / metabolism
  • Humans
  • Lipid Metabolism*
  • Mitochondria / metabolism
  • Oxidation-Reduction
  • Peroxisomal Disorders / genetics
  • Peroxisomal Disorders / metabolism*
  • Peroxisomes / physiology*
  • Phospholipid Ethers / metabolism
  • Racemases and Epimerases / deficiency


  • Fatty Acids
  • Phospholipid Ethers
  • Acyl-CoA Oxidase
  • Racemases and Epimerases
  • alpha-methylacyl-CoA racemase