The role of the carnitine system in peroxisomal fatty acid oxidation.
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 alpha-oxidation for phytanic acid and beta-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.
School of Biomedical Sciences, University of St. Andrews, Fife, UK. rrr@st-and.ac.uk
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D000818:Animals; D002331:Carnitine / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D015227:Lipid Peroxidation; D008830:Microbodies / Q000378:metabolism*; D010084:Oxidation-Reduction
D005227:Fatty Acids; D002331:Carnitine