

| Catalog No. | HY508014 | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| Species reactivity | Human, Mouse, Rat | ||||||||
| Applications | ELISA, IHC, WB | ||||||||
| Host species | Rabbit | ||||||||
| Clonality | Polyclonal | ||||||||
| Isotype | IgG | ||||||||
| Immunogen | E. coli - derived recombinant Human PCCA (Val580-Glu728). | ||||||||
| Target | PCCase subunit alpha, Propionyl-CoA carboxylase alpha chain, mitochondrial, Propanoyl-CoA:carbon dioxide ligase subunit alpha, PCCA | ||||||||
| Purification | Purified by antigen affinity column. | ||||||||
| Accession | P05165 | ||||||||
| Form | Liquid | ||||||||
| Storage buffer | 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. Please refer to the specific buffer information in the hardcopy of datasheet or the lot-specific COA. |
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| Product Usage Information |
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| Stability and Storage | Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt. | ||||||||
| Background | Propionyl-CoA carboxylase alpha chain, mitochondrial (PCCA) is a ~80 kDa protein. 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. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. 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. 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. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA. 1. Kalousek, F. et al. (1980) The Journal of biological chemistry 255, 60-5. PMID: 6765947 2. Stankovics, J. et al. (1993) American journal of human genetics 52, 144-51. PMID: 8434582 3. Richard, E. et al. (1999) Biochimica et biophysica acta 1453, 351-8. PMID: 10101253 | ||||||||
| Note | For research use only. |
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