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R Amino acid conjugation: The two-step reaction involves: (1) mitochondrial medium-chain acyl-CoA ligase (HXM-A and HXM-B) activating the carboxylic acid substrate with CoA in an ATP-dependent reaction to form an acyl-CoA thioester, and (2) GLYAT (glycine N-acyltransferase) transferring the acyl group from CoA to the alpha-amino group of glycine via a ping-pong mechanism

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