PCCA Antibody (D-5): sc-374341
- PCCA Antibody (D-5) is a mouse monoclonal IgG1 κ PCCA antibody, cited in 1 publications, provided at 200 µg/ml
- specific for an epitope mapping between amino acids 195-231 within an internal region of PCCA of human origin
- PCCA Antibody (D-5) is recommended for detection of PCCA of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA
- Anti-PCCA Antibody (D-5) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- m-IgG Fc BP-HRP and m-IgG1 BP-HRP are the preferred secondary detection reagents for PCCA Antibody (D-5) for WB and IHC(P) applications. These reagents are now offered in bundles with PCCA Antibody (D-5) (see ordering information below).
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SEE ALSO...
PCCA Antibody (D-5) is a mouse monoclonal IgG1 kappa light chain antibody that detects PCCA protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). PCCA (D-5) antibody is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. PCCA protein, or propionyl Coenzyme A carboxylase alpha polypeptide, is a crucial 703 amino acid enzyme localized in the mitochondrial matrix, where PCCA plays a vital role in the catabolism of branched-chain amino acids, odd-chain fatty acids, and other metabolites. This enzyme is biotin-dependent and forms carboxybiotin through ATP hydrolysis, featuring a C-terminal biotin-binding domain and a biotin carboxylase domain. Proper functioning of PCCA is essential for metabolic processes, as inherited mutations in the gene encoding PCCA can lead to propionic acidemia, a serious autosomal recessive disorder that disrupts the catabolism of specific amino acids and odd-numbered chain fatty acids. PCCA exists in two isoforms due to alternative splicing, which may have implications for functional diversity and regulation in metabolic pathways.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
PCCA Antibody (D-5) References:
- Overview of mutations in the PCCA and PCCB genes causing propionic acidemia. | Ugarte, M., et al. 1999. Hum Mutat. 14: 275-82. PMID: 10502773
- Functional characterization of PCCA mutations causing propionic acidemia. | Clavero, S., et al. 2002. Biochim Biophys Acta. 1588: 119-25. PMID: 12385775
- Molecular analysis of PCCB gene in Korean patients with propionic acidemia. | Kim, SN., et al. 2002. Mol Genet Metab. 77: 209-16. PMID: 12409268
- Mutation spectrum of the PCCA and PCCB genes in Japanese patients with propionic acidemia. | Yang, X., et al. 2004. Mol Genet Metab. 81: 335-42. PMID: 15059621
- Propionic acidemia: mutation update and functional and structural effects of the variant alleles. | Desviat, LR., et al. 2004. Mol Genet Metab. 83: 28-37. PMID: 15464417
- New splicing mutations in propionic acidemia. | Desviat, LR., et al. 2006. J Hum Genet. 51: 992-997. PMID: 17051315
- High frequency of large genomic deletions in the PCCA gene causing propionic acidemia. | Desviat, LR., et al. 2009. Mol Genet Metab. 96: 171-6. PMID: 19157943
- Chaperonin-mediated assembly of wild-type and mutant subunits of human propionyl-CoA carboxylase expressed in Escherichia coli. | Kelson, TL., et al. 1996. Hum Mol Genet. 5: 331-7. PMID: 8852656
- Three novel splice mutations in the PCCA gene causing identical exon skipping in propionic acidemia patients. | Richard, E., et al. 1997. Hum Genet. 101: 93-6. PMID: 9385377
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
PCCA Antibody (D-5) | sc-374341 | 200 µg/ml | $322.00 | |||
PCCA Antibody (D-5): m-IgG Fc BP-HRP Bundle | sc-527452 | 200 µg Ab; 10 µg BP | $361.00 | |||
PCCA Antibody (D-5): m-IgG1 BP-HRP Bundle | sc-532825 | 200 µg Ab; 20 µg BP | $361.00 | |||
PCCA Antibody (D-5) AC | sc-374341 AC | 500 µg/ml, 25% agarose | $424.00 | |||
PCCA Antibody (D-5) HRP | sc-374341 HRP | 200 µg/ml | $322.00 | |||
PCCA Antibody (D-5) FITC | sc-374341 FITC | 200 µg/ml | $336.00 | |||
PCCA Antibody (D-5) PE | sc-374341 PE | 200 µg/ml | $349.00 | |||
PCCA Antibody (D-5) Alexa Fluor® 488 | sc-374341 AF488 | 200 µg/ml | $364.00 | |||
PCCA Antibody (D-5) Alexa Fluor® 546 | sc-374341 AF546 | 200 µg/ml | $364.00 | |||
PCCA Antibody (D-5) Alexa Fluor® 594 | sc-374341 AF594 | 200 µg/ml | $364.00 | |||
PCCA Antibody (D-5) Alexa Fluor® 647 | sc-374341 AF647 | 200 µg/ml | $364.00 | |||
PCCA Antibody (D-5) Alexa Fluor® 680 | sc-374341 AF680 | 200 µg/ml | $364.00 | |||
PCCA Antibody (D-5) Alexa Fluor® 790 | sc-374341 AF790 | 200 µg/ml | $364.00 | |||
PCCA (D-5) Neutralizing Peptide | sc-374341 P | 100 µg/0.5 ml | $69.00 |