Anti-AMACR Antibody Picoband® Fluoro647 Conjugated

Alpha-methylacyl-CoA racemase (AMACR) is a mitochondrial and peroxisomal enzyme. It encodes a racemase. The encoded enzyme 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. Encoded proteins from this locus localize to both mitochondria and peroxisomes. Mutations in this gene 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 this gene and the upstream neighboring C1QTNF3 (C1q and tumor necrosis factor related protein 3) gene.

A synthetic peptide corresponding to a sequence in the middle region of human AMACR, different from the related mouse and rat sequences by four amino acids.

Widely expressed. Expressed at higher level in thymus (medullary epithelial cells and monocyte-dendritic cells), pancreas, adrenal cortex and testis. Expressed at lower level in the spleen, fetal liver and lymph nodes. Isoform 2 and isoform 3 seem to be less frequently expressed than isoform 1, if at all.

Cancer, Cancer Metabolism, Cancer Susceptibility, Cell Type Markers, Epigenetics and Nuclear Signaling, Lipid and Lipoprotein Metabolism, Lipid Metabolism, Metabolic Signaling Pathway, Metabolic Signaling Pathways, Metabolism, Metabolism of Lipids and Lipoproteins, Mitochondrial Markers, Mitochondrial Metabolism, Oxidative Stress, Pathways and Processes, Proto-Oncogenes, Redox Metabolism, Signal Transduction, Tags & Cell Markers, Transcription, Tumor Associated

Racemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S) -stereoisomers.

1. Amery, L., Fransen, M., De Nys, K., Mannaerts, G. P., Van Veldhoven, P. P. Mitochondrial and peroxisomal targeting of 2-methylacyl-CoA racemase in humans. J. Lipid Res. 41: 1752-1759, 2000. 2. Clarke, C. E., Alger, S., Preece, M. A., Burdon, M. A., Chavda, S., Denis, S., Ferdinandusse, S., Wanders, R. J. A. Tremor and deep white matter changes in alpha-methylacyl-CoA racemase deficiency. Neurology 63: 188-189, 2004. 3. Dick, D., Horvath, R., Chinnery, P. F. AMACR mutations cause late-onset autosomal recessive cerebellar ataxia. Neurology 76: 1768-1770, 2011.