AMACR Monoclonal Antibody

AMACR encodes a racemase. Alpha-methylacyl-CoA racemase interconverts pristanoyl-CoA and C27-bile acylCoAs between their (R)- and (S)-stereoisomers. The conversion to the (S)-stereoisomers is necessary for degradation of these substrates by peroxisomal beta-oxidation. Alpha-methylacyl-CoA racemase from this locus localize to both mitochondria and peroxisomes. Mutations in AMACR may be associated with adult-onset sensorimotor neuropathy, pigmentary retinopathy, and adrenomyeloneuropathy due to defects in bile acid synthesis. Alternatively spliced transcript variants have been described. Read-through transcription also exists between AMACR and the upstream neighboring C1QTNF3 (C1q and tumor necrosis factor related protein 3) gene.