Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.05% Sodium azide
Constituent: 1% BSA
Concentration information loading...
Purification notesPurified immunoglobulin fraction of rabbit antiserum
- Pathways and Processes
- Metabolic signaling pathways
- Lipid and lipoprotein metabolism
- Lipid metabolism
Our Abpromise guarantee covers the use of ab12498 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Not tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
FunctionRacemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers.
PathwayLipid metabolism; bile acid biosynthesis.
Lipid metabolism; fatty acid metabolism.
Involvement in diseaseDefects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:614307]. AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. It can be associated with polyneuropathy, retinitis pigmentosa, epilepsy.
Defects in AMACR are the cause of congenital bile acid synthesis defect type 4 (CBAS4) [MIM:214950]; also known as cholestasis, intrahepatic, with defective conversion of trihydroxycoprostanic acid to cholic acid or trihydroxycoprostanic acid in bile. Clinical features include neonatal jaundice, intrahepatic cholestasis, bile duct deficiency and absence of cholic acid from bile.
Sequence similaritiesBelongs to the CaiB/BaiF CoA-transferase family.
Cellular localizationPeroxisome. Mitochondrion.
- Information by UniProt
- 2 arylpropionyl CoA epimerase antibody
- 2 methylacyl CoA racemase antibody
- 2-methylacyl-CoA racemase antibody
ab12498 has not yet been referenced specifically in any publications.