Specifications
- Antibody type:Primary
- Antigen name:alpha-methylacyl-CoA racemase
- Antigen symbol:AMACR
- Clonality:Polyclonal
- Conjugation:Unconjugated
- Host:Rabbit
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Western blot:Yes
- Cross adsorption:No
- Form:Lyophilized
- Gene ID:23600
- Antigen synonyms:AMACRD|RM|RACE|CBAS4
- Storage temperature:At -20˚C for one year. After reconstitution, at 4˚C for one month. It can also be aliquotted and stored frozen at -20˚C for a longer time. Avoid repeated freezing and thawing.
- Immunogen:A synthetic peptide corresponding to a sequence in the middle region of human AMACR (208-246aa RGQNMLDGGAPFYTTYRTADGEFMAVGAIEPQFYELLIK), different from the related mouse and rat sequences by four amino acids.
- Size:100 μg
- Pk:100 µG
Specifications
About this item
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.
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.
Add 0.2ml of distilled water will yield a concentration of 500ug/ml.