Rabbit Polyclonal PCCA antibody. Suitable for WB, IHC-P and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human PCCA aa 450 to C-terminus.
Images
Publications
Filter by
- Orphanet journal of rare diseases 17:622022Therapeutic potential of living donor liver transplantation from heterozygous carrier donors in children with propionic acidemia.Applications:Unspecified applicationReactive species:Unspecified reactive speciesZhi-Gui Zeng,Guang-Peng Zhou,Lin Wei,Wei Qu,Ying Liu,Yu-Le Tan,Jun Wang,Li-Ying Sun,Zhi-Jun ZhuPubMed 35189944
Key facts
- Isotype
- IgG
- Host species
- Rabbit
- Storage buffer
pH: 7
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 78.99% PBS, 20% Glycerol (glycerin, glycerine), 1% BSA- Form
- Liquid
- Clonality
- Polyclonal
Immunogen
- Recombinant Fragment Protein within Human PCCA aa 450 to C-terminus. The exact immunogen used to generate this antibody is proprietary information. Database link P05165
Reactivity data
Select an application| WB | IHC-P | |
|---|---|---|
| Human | Tested | Tested |
WB
TestedTested
| Species | Dilution info | Notes |
|---|---|---|
Species Human | Dilution info 1/1000.00000 - 1/10000.00000 | Notes - |
IHC-P
TestedTested
| Species | Dilution info | Notes |
|---|---|---|
Species Human | Dilution info 1/100.00000 - 1/1000.00000 | Notes - |
Associated Products
Select an associated product type
2 products for Alternative Product
Target data
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).
Alternative names
PCCase subunit alpha, Propanoyl-CoA:carbon dioxide ligase subunit alpha, PCCA
Recommended products
Rabbit Polyclonal PCCA antibody. Suitable for WB, IHC-P and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human PCCA aa 450 to C-terminus.
Key facts
- Isotype
- IgG
- Host species
- Rabbit
- Storage buffer
pH: 7
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 78.99% PBS, 20% Glycerol (glycerin, glycerine), 1% BSA- Form
- Liquid
- Clonality
- Polyclonal
- Immunogen
- Recombinant Fragment Protein within Human PCCA aa 450 to C-terminus. The exact immunogen used to generate this antibody is proprietary information. Database link P05165
- Purification technique
- Affinity purification Immunogen
- Concentration
Storage
- Shipped at conditions
- Blue Ice
- Appropriate short-term storage conditions
- +4°C
- Appropriate long-term storage conditions
- -20°C
- Aliquoting information
- Upon delivery aliquot
- Storage information
- Avoid freeze / thaw cycle
Notes
Abcam is leading the way to address reproducibility in scientific research with our highly validated recombinant monoclonal and recombinant multiclonal antibodies. Search & select one of Abcam's thousands of recombinant alternatives to eliminate batch-variability and unnecessary animal use.
If you do not find a host species to meet your needs, our catalogue and custom Chimeric range provides scientists the specificity of Abcam's RabMAbs in the species backbone of your choice. Remember to also review our range of edited cell lines, proteins and biochemicals relevant to your target that may help you further your research goals.
Abcam antibodies are extensively validated in a wide range of species and applications, so please check the reagent specifications meet your scientific needs before purchasing. If you have any questions or bespoke requirements, simply visit the Contact Us page to send us an inquiry or contact our Support Team ahead of purchase.
Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
PCCA also known as propionyl-CoA carboxylase alpha chain is an essential enzyme component in humans. It has a molecular mass of approximately 81.4 kDa and is primarily expressed in the liver and kidney. PCCA forms a biotin-dependent enzyme that catalyzes the carboxylation of propionyl-CoA to form D-methylmalonyl-CoA. This action is an important step in the mitochondrial beta-oxidation of odd-chain fatty acids and some amino acids.
- Biological function summary
PCCA is a part of the propionyl-CoA carboxylase complex which functions as a heterododecameric complex involving both alpha and beta subunits. This enzyme plays an important role in energy metabolism facilitating the conversion of propionyl-CoA into more useful forms for cellular processes. The proper functioning of this complex ensures the breakdown and assimilation of important metabolites aiding in the elimination of toxic intermediates produced during the metabolism of branched-chain amino acids as well as odd-chain fatty acids.
- Pathways
The catabolism facilitated by PCCA involves the metabolic pathways of branched-chain amino acids and odd-numbered fatty acids. It fits within the larger methylmalonyl-CoA mutase pathway where it transforms its substrate into an immediate precursor for succinyl-CoA production. The activity in this pathway links PCCA to other proteins such as methylmalonyl-CoA mutase which subsequently acts to convert methylmalonyl-CoA to succinyl-CoA a critical step for entry into the tricarboxylic acid (TCA) cycle.
- Associated diseases and disorders
PCCA defects are linked to propionic acidemia a serious metabolic disorder characterized by the accumulation of propionic acid. This disease results from genetic mutations affecting the normal function of the PCCA protein leading to toxic buildup of metabolites in blood and tissues. The disorder can cause severe metabolic acidosis and can impact neurological development. Additionally PCCA interacts with various cellular proteins such as methylmalonyl-CoA mutase which if disrupted also associates with metabolic disorders exemplifying the interdependent relationship between components in metabolic pathways.
Product promise
Tested
We have tested this species and application combination and it works. It is covered by our product promise.
Expected
We have not tested this specific species and application combination in-house, but expect it will work. It is covered by our product promise.
Predicted
This species and application combination has not been tested, but we predict it will work based on strong homology. However, this combination is not covered by our product promise.
Not recommended
We do not recommend this combination. It is not covered by our product promise.
We are dedicated to supporting your work with high quality reagents and we are here for you every step of the way should you need us.
In the unlikely event of one of our products not working as expected, you are covered by our product promise.
Full details and terms and conditions can be found here:
Terms & Conditions.
2 product images
Western blot - Anti-PCCA antibody (ab154254)
7.5% SDS PAGE
All lanes: Western blot - Anti-PCCA antibody (ab154254) at 1/5000 dilution
All lanes: HeLa whole cell lysate at 30 µg
Predicted band size: 80 kDa
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PCCA antibody (ab154254)
Immunohistochemical analysis of paraffin-embedded U87 xenograft tissue labeling PCCA with ab154254 at 1/500 dilution.
Downloads
Product protocols
- Visit the general protocols
- Visit troubleshooting
Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.
For licensing inquiries, please contact partnerships@abcam.com