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AB154254

Anti-PCCA antibody

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(1 Publication)

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.

View Alternative Names

PCCase subunit alpha, Propanoyl-CoA:carbon dioxide ligase subunit alpha, PCCA

2 Images
Western blot - Anti-PCCA antibody (AB154254)
  • WB

Unknown

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

false

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PCCA antibody (AB154254)
  • IHC-P

Unknown

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.

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

No

Reacts with

Human

Applications

WB, IHC-P

applications

Immunogen

Recombinant Fragment Protein within Human PCCA aa 450 to C-terminus. The exact immunogen used to generate this antibody is proprietary information.

P05165

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/1000.00000 - 1/10000.00000", "WB-species-notes": "<p></p>", "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/100.00000 - 1/1000.00000", "IHCP-species-notes": "<p></p>" } } }
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We recommend this product because it’s often used in the same experiment or related research.

We advise that you always check the datasheet to ensure it fits your experiments, or contact ourtechnical teamfor help.

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Properties and storage information

Form
Liquid
Purification technique
Affinity purification Immunogen
Storage buffer
pH: 7 Preservative: 0.01% Thimerosal (merthiolate) Constituents: 78.99% PBS, 20% Glycerol (glycerin, glycerine), 1% BSA
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
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

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.

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.
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Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:
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Target data

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).
See full target information PCCA
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Publications (1)

Recent publications for all applications. Explore the full list and refine your search

Orphanet journal of rare diseases 17:62 PubMed35189944

2022

Therapeutic potential of living donor liver transplantation from heterozygous carrier donors in children with propionic acidemia.

Applications

Unspecified application

Species

Unspecified reactive species

Zhi-Gui Zeng,Guang-Peng Zhou,Lin Wei,Wei Qu,Ying Liu,Yu-Le Tan,Jun Wang,Li-Ying Sun,Zhi-Jun Zhu
View all publications
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Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.
For full details, please see our Terms & Conditions
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Associated Products

Select an associated product type
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