Peroxisomes, lipid metabolism, and peroxisomal disorders.
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.
Wanders
R J A
RJ
Laboratory for Genetic Metabolic Diseases, Department of Clinical Chemistry and Pediatrics, Academic Medical Center, University of Amsterdam, Emma Children's Hospital, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl
D042962:Acyl-CoA Oxidase / Q000172:deficiency; D001692:Biological Transport; D005227:Fatty Acids / Q000378:metabolism; D006801:Humans; D050356:Lipid Metabolism; D008928:Mitochondria / Q000378:metabolism; D010084:Oxidation-Reduction; D018901:Peroxisomal Disorders / Q000235:genetics / Q000378:metabolism*; D020675:Peroxisomes / Q000502:physiology*; D010742:Phospholipid Ethers / Q000378:metabolism; D019998:Racemases and Epimerases / Q000172:deficiency
D005227:Fatty Acids; D010742:Phospholipid Ethers; D042962:Acyl-CoA Oxidase; D019998:Racemases and Epimerases; C108352:alpha-methylacyl-CoA racemase