|Other Names||Alpha-methylacyl-CoA racemase, 2-methylacyl-CoA racemase, AMACR|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|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.|
|Cellular Location||Peroxisome. Mitochondrion|
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This gene encodes a racemase. The encoded enzymeinterconverts pristanoyl-CoA and C27-bile acylCoAs between their(R)- and (S)-stereoisomers. The conversion to the (S)-stereoisomersis necessary for degradation of these substrates by peroxisomalbeta-oxidation. Encoded proteins from this locus localize to bothmitochondria and peroxisomes. Mutations in this gene may beassociated with adult-onset sensorimotor neuropathy, pigmentaryretinopathy, and adrenomyeloneuropathy due to defects in bile acidsynthesis. Alternatively spliced transcript variants have beendescribed.
Murray, N.P., et al. Oncol. Rep. 24(3):687-692(2010)Sonwalkar, S.A., et al. Histopathology 56(7):900-907(2010)Lakis, S., et al. World J. Gastroenterol. 16(20):2476-2483(2010)Chen, W., et al. Mol. Biol. Rep. 36(3):423-430(2009)Mubiru, J.N., et al. Gene 327(1):89-98(2004)
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