Anti-PCCB antibody

Supplementary info

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

Activity summary

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.

Biological function summary

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.

Pathways

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.

Associated diseases and disorders

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.