Abcam, ab187686, Anti-PCCA antibody - C-terminal

Size: 100µg
Rabbit Polyclonal PCCA antibody. C-terminal. Suitable for WB, IHC-Fr and reacts with Human, Mouse samples. Cited in 8 publications. Immunogen corresponding to Synthetic Peptide within Human PCCA aa 650 to C-terminus.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Mouse, Human,
Applications:IHC-Fr, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Synthetic Peptide within Human PCCA aa 650 to C-terminus. The exact immunogen used to generate this antibody is proprietary information.P05165

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Purification notes-ab187686 was affinity purified using an epitope specific to PCCA immobilized on solid support., Storage buffer-pH: 7 - 8Preservative: 0.09% Sodium azideConstituents: 99% Tris citrate/phosphate, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions-+4°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

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.