Peroxisomal fatty acid alpha- and beta-oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal diseases

Biochem Soc Trans. 2001 May;29(Pt 2):250-67. doi: 10.1042/0300-5127:0290250.


Peroxisomes are subcellular organelles with an indispensable role in cellular metabolism. The importance of peroxisomes for humans is stressed by the existence of a group of genetic diseases in humans in which there is an impairment in one or more peroxisomal functions. Most of these functions have to do with lipid metabolism including the alpha- and beta-oxidation of fatty acids. Here we describe the current state of knowledge about peroxisomal fatty acid alpha- and beta-oxidation with particular emphasis on the following: (1) the substrates beta-oxidized in peroxisomes; (2) the enzymology of the alpha- and beta-oxidation systems; (3) the permeability properties of the peroxisomal membrane and the role of the different transporters therein; (4) the interaction with other subcellular compartments, including the mitochondria, which are the ultimate site of NADH re-oxidation and full degradation of acetyl-CoA to CO(2) and water; and (5) the different disorders of peroxisomal alpha- and beta-oxidation.

Publication types

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

MeSH terms

  • Adenosine Triphosphate / metabolism
  • Animals
  • Biological Transport
  • Carbon Dioxide / metabolism
  • Carnitine / metabolism
  • Carnitine O-Palmitoyltransferase / metabolism
  • Fatty Acids / chemistry
  • Fatty Acids / metabolism*
  • Humans
  • Intracellular Membranes / metabolism
  • Mitochondria / enzymology
  • Mitochondria / metabolism
  • Mutation / genetics
  • Peroxisomal Disorders / enzymology*
  • Peroxisomal Disorders / genetics
  • Peroxisomal Disorders / metabolism*
  • Peroxisomes / enzymology*
  • Peroxisomes / metabolism*
  • Racemases and Epimerases / deficiency
  • Racemases and Epimerases / genetics
  • Racemases and Epimerases / metabolism
  • Substrate Specificity
  • Water / metabolism


  • Fatty Acids
  • Water
  • Carbon Dioxide
  • Adenosine Triphosphate
  • Carnitine O-Palmitoyltransferase
  • Racemases and Epimerases
  • alpha-methylacyl-CoA racemase
  • Carnitine