|Other Names||Propionyl-CoA carboxylase alpha chain, mitochondrial, PCCase subunit alpha, Propanoyl-CoA:carbon dioxide ligase subunit alpha, PCCA|
|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.|
|Cellular Location||Mitochondrion matrix|
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Provided below are standard protocols that you may find useful for product applications.
The protein encoded by this gene is the alpha subunit ofthe heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase.PCCA encodes the biotin-binding region of this enzyme. Mutations ineither PCCA or PCCB (encoding the beta subunit) lead to an enzymedeficiency resulting in propionic acidemia. Multiple transcriptvariants encoding different isoforms have been found for this gene.
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)Huang, C.S., et al. Nature 466(7309):1001-1005(2010)Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)MacDonald, M.J., et al. Diabetologia 52(6):1087-1091(2009)
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