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Correspondence to:
Ronald J. A. Wanders
Both in vitro studies with purified heterologously expressed protein and in vivo studies in fibroblasts of patients with an
Supplementary key words:
branched-chain fatty acid ß-oxidation, stereospecificity, (2R,6)-dimethylheptanoyl-CoA
Copyright © 2000 by Lipid Research, Inc.
Original Article
Subcellular localization and physiological role of
Sacha Ferdinandussea,
Simone Denisa,
Lodewijk IJlsta,
Georges Dacremontc,
Hans R. Waterhamb, and
Ronald J. A. Wandersa,b
-methylacyl-CoA racemase
a Departments of Clinical Chemistry, Emma Children's Hospital, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands
b Pediatrics, Emma Children's Hospital, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands
c Department of Pediatrics, University of Ghent, 9000 Ghent, Belgium
-Methylacyl-CoA racemase plays an important role in the ß-oxidation of branched-chain fatty acids and fatty acid derivatives because it catalyzes the conversion of several (2R)-methyl-branched-chain fatty acyl-CoAs to their (S)-stereoisomers. Only stereoisomers with the 2-methyl group in the (S)-configuration can be degraded via ß-oxidation. Patients with a deficiency of -methylacyl-CoA racemase accumulate in their plasma pristanic acid and the bile acid intermediates di- and trihydroxycholestanoic acid, which are all substrates of the peroxisomal ß-oxidation system. Subcellular fractionation experiments, however, revealed that both in humans and rats -methylacyl-CoA racemase is bimodally distributed to both the peroxisome and the mitochondrion. Our findings show that the peroxisomal and mitochondrial enzymes are produced from the same gene and that, as a consequence, the bimodal distribution pattern must be the result of differential targeting of the same gene product. In addition, we investigated the physiological role of the enzyme in the mitochondrion. -methylacyl-CoA racemase deficiency revealed that the mitochondrial enzyme plays a crucial role in the mitochondrial ß-oxidation of the breakdown products of pristanic acid by converting (2R,6)-dimethylheptanoyl-CoA to its (S)-stereoisomer. — Ferdinandusse, S., S. Denis, L. IJlst, G. Dacremont, H. R. Waterham, and R. J. A. Wanders. Subcellular localization and physiological role of -methylacyl-CoA racemase. J. Lipid Res. 2000. 41: 1890;–1896. 
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