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PCCA Antibody (Center)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, IHC-P, E |
|---|---|
| Primary Accession | P05165 |
| Other Accession | NP_001121164.1, NP_000273.2 |
| Reactivity | Human, Rat |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | Rabbit IgG |
| Calculated MW | 80059 Da |
| Antigen Region | 362-390 aa |
| Gene ID | 5095 |
|---|---|
| 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. |
| Dilution | WB~~1:2000 IHC-P~~1:25 |
| 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. |
| Name | PCCA (HGNC:8653) |
|---|---|
| Function | This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites (PubMed:6765947, PubMed:8434582). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA (PubMed:10101253, PubMed:6765947, PubMed:8434582). Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity). Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)- ethylmalonyl-CoA at a much lower rate (PubMed:6765947). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity). |
| Cellular Location | Mitochondrion matrix |
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Provided below are standard protocols that you may find useful for product applications.
Background
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.
References
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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