| Product name: |
P504S rabbit pAb |
| Reactivity: |
Human;Mouse;Rat |
| Alternative Names: |
Alpha-methylacyl-CoA racemase (EC 5.1.99.4;2-methylacyl-CoA racemase) |
| Source: |
Rabbit |
| Dilutions: |
WB 1:1000-2000 ELISA 1:5000-20000 |
| Immunogen: |
Synthesized peptide derived from human P504S AA range: 271-320 |
| Storage: |
-20C/1 year |
| Clonality: |
Polyclonal |
| Isotype: |
IgG |
| Concentration: |
1 mg/ml |
| Molecular Weight: |
42kD |
| GeneID: |
23600 |
| Human Swiss-Prot No: |
Q9UHK6 |
| Cellular localization: |
Peroxisome . Mitochondrion . |
| Background: |
This gene 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. [provided by RefSeq, Mar 2011], |
