|Application ||IHC-P, WB, E|
|Other Accession||NP_001121164.1, NP_000273.2|
|Calculated MW||80059 Da|
|Antigen Region||362-390 aa|
|Other Names||Propionyl-CoA carboxylase alpha chain, mitochondrial, PCCase subunit alpha, Propanoyl-CoA:carbon dioxide ligase subunit alpha, PCCA|
|Target/Specificity||This PCCA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 362-390 amino acids from the Central region of human PCCA.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||PCCA Antibody (Center) is for research use only and 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 of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants 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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