Alpha-methylacyl-CoA racemase

Definition:

Catalyzes the interconversion of (R)- and (S)-stereoisomers of alpha-methyl-branched-chain fatty acyl-CoA esters. Acts only on coenzyme A thioesters, not on free fatty acids, and accepts as substrates a wide range of alpha-methylacyl-CoAs, including pristanoyl-CoA, trihydroxycoprostanoyl-CoA (an intermediate in bile acid synthesis), and arylpropionic acids like the anti-inflammatory drug ibuprofen (2-(4-isobutylphenyl)propionic acid) but neither 3-methyl-branched nor linear-chain acyl-CoAs.

References:

[1]. S Ferdinandusse, et al. Subcellular localization and physiological role of alpha-methylacyl-CoA racemase. J Lipid Res. 2000 Nov;41(11):1890-6. [Content Brief]

[2]. W Schmitz, et al. Purification and characterization of an alpha-methylacyl-CoA racemase from human liver. Eur J Biochem. 1995 Aug 1;231(3):815-22. [Content Brief]

[3]. S Ferdinandusse, et al. Mutations in the gene encoding peroxisomal alpha-methylacyl-CoA racemase cause adult-onset sensory motor neuropathy. Nat Genet. 2000 Feb;24(2):188-91. [Content Brief]

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