- Catalog number
- PB9983
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- 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.
- Synonyms
- Alpha-methylacyl-CoA racemase;5.1.99.4;2-methylacyl-CoA racemase; AMACR
- Gene Name
- AMACR
- Gene ID
- 23600
- UniProt
- Q9UHK6
- Host
- Rabbit
- Reactivity
- Human, Mouse, Rat
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- 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.
- Clonality
- Polyclonal
- Tissue Specificity
- 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.
- Applications
- Flow Cytometry, IF, IHC, ICC, WB
- Field of Research
- 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
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- 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.
- References & Citations
- 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.
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 42387 MW
- Observed Molecular Weight
- 42 kDa
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- 6
- Gene Name Synonym
- Alpha-methylacyl-CoA racemase
- Subcellular Location
- Peroxisome . Mitochondrion .
- Protein Name
- Alpha-methylacyl-CoA racemase
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.