SUMMARY, EXPLANATION AND LIMITATIONS:
p504S, also known as AMACR (α-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 β 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, and/or CBAS4 (congenital bile acid synthesis defect type 4), a disorder characterized by intrahepatic cholestasis, absence of cholic acid from bile, neonatal jaundice and bile duct deficiency. In addition, p504S is overexpressed in prostate cancer and is believed to be functionally important for prostate cancer cell growth.
Immunogen: Purified recombinant fusion protein AMACR-His expressed in E. Lineage coli BL21 (DE3).
Staining pattern: Cytoplasmic.
Positive control: Tissue sample from prostate carcinoma.
This antibody is designed for the specific localization of human p504S using IHC techniques in formalin-fixed, paraffin-embedded tissue sections.