AMACR (C-terminus) monoclonal antibody

P504S, also known as AMACR (a-methylacyl-CoA racemase), 2-methylacyl-CoA racemase or RACE, is an enzyme belonging to the caiB/baiF CoA-transferase family. Localizing to the peroxisome and mitochondrion, P504S plays a role in the metabolism of branched-chain fatty acids and bile acid intermediates. More specifically, P504S catalyzes the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers which can then be degraded by peroxisomal b-oxidation. Mutations in the gene encoding P504S can lead to AMACR deficiency, a disease characterized by increased concentrations of pristanic acid that is associated with adult onset sensory motor neuropathy.