Recombinant Human PCCB protein is a Human Fragment protein, in the 7 to 266 aa range, expressed in Escherichia coli, with =90% purity and suitable for SDS-PAGE.
=90% SDS-PAGE
Escherichia coli
Tag free
SDS-PAGE
No
Application | Reactivity | Dilution info | Notes |
---|---|---|---|
Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
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:15890657, PubMed:6765947). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:15890657, PubMed:6765947). 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).
PCCase subunit beta, Propanoyl-CoA:carbon dioxide ligase subunit beta, PCCB
Recombinant Human PCCB protein is a Human Fragment protein, in the 7 to 266 aa range, expressed in Escherichia coli, with =90% purity and suitable for SDS-PAGE.
=90% SDS-PAGE
Escherichia coli
Tag free
SDS-PAGE
No
No
Human
Reconstitute in water
Constituents: 0.58% Sodium chloride, 0.32% Tris HCl
Fragment
27.57 kDa
7 to 266
Recombinant
Lyophilized
Purified via His tag
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:15890657, PubMed:6765947). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:15890657, PubMed:6765947). 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).
Belongs to the AccD/PCCB family.
Mitochondrion matrix
Blue Ice
-20°C
-20°C
Upon delivery aliquot
Avoid freeze / thaw cycle
This supplementary information is collated from multiple sources and compiled automatically.
PCCB also known as Propionyl-CoA Carboxylase β subunit is an enzyme component that plays an important role in metabolizing certain amino acids and odd-chain fatty acids. This protein partners with the alpha subunit to form a dimeric enzyme with a total mass of approximately 750 kDa. PCCB is mainly expressed in the liver but its expression also occurs in other tissues such as the kidney and heart where energy and metabolism play key roles.
Propionyl-CoA Carboxylase β subunit takes part in the process of carboxylation of propionyl-CoA to methylmalonyl-CoA an essential step in catabolism. This subunit contributes to the function of the complex necessary for efficient energy production and proper detoxification of specific intermediary products. In this enzymatic action it works jointly with the alpha subunit that harbors the biotin-binding sites important for transferring carbon dioxide.
PCCB is an important component in the propionic acid pathway and is critical for maintaining the balance in mitochondrial fatty acid synthesis. Its function plays a central role in the catabolism of certain amino acids including valine isoleucine and methionine as well as odd-chain fatty acids. Propionyl-CoA Carboxylase β subunit’s action works closely with methylmalonyl-CoA mutase assisting in metabolic conversion that takes place after the initial carboxylation step.
Deficiencies or mutations in PCCB are linked to propionic acidemia a metabolic disorder that interrupts normal propionic acid breakdown leading to an accumulation that is toxic to the body. This deficiency relates it to methylmalonic acidemia if methylmalonyl-CoA mutase is affected often causing similar clinical symptoms such as developmental delays vomiting and metabolic crises. Understanding the function and pathways of PCCB helps to develop targeted therapies that address its metabolic roles and its involvement in these disorders.
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.
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