α-Methylacyl-CoA racemase (AMACR, EC 5.1.99.4) is an enzyme that in humans is encoded by the AMACR gene.[5][6][7] AMACR catalyzes the following chemical reaction:
- (2R)-2-methylacyl-CoA (2S)-2-methylacyl-CoA
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In mammalian cells, the enzyme is responsible for converting (2R)-methylacyl-CoA esters to their (2S)-methylacyl-CoA epimers and known substrates, including coenzyme A esters of pristanic acid (mostly derived from phytanic acid, a 3-methyl branched-chain fatty acid that is abundant in the diet) and bile acids derived from cholesterol. This transformation is required in order to degrade (2R)-methylacyl-CoA esters by β-oxidation, which process requires the (2S)-epimer. The enzyme is known to be localised in peroxisomes and mitochondria, both of which are known to β-oxidize 2-methylacyl-CoA esters.[8][9]