COASY Recombinant Rabbit Monoclonal Antibody [JE58-56] (HA721128)

UNIPROT #

Q13057

PROTEIN NAME

COASY

SYNONYMS

Bifunctional coenzyme A synthase antibody;Bifunctional phosphopantetheine adenylyl transferase/dephospho CoA kinase antibody;CoA synthase antibody;COASY antibody;COASY_HUMAN antibody;Coenzyme A synthase antibody;Dephospho-CoA kinase antibody;Dephospho-CoA pyrophosphorylase antibody;Dephosphocoenzyme A kinase antibody;DPCK antibody;DPCOAK antibody;NBP antibody;Nucleotide binding protein antibody;Pantetheine-phosphate adenylyltransferase antibody;Phosphopantetheine adenylyltransferase / dephosphocoenzyme A kinase antibody;pOV 2 antibody;POV-2 antibody;POV2 antibody;PPAT antibody;UKR1 antibody

SUBCELLULAR LOCATION

Mitochondrion matrix, Cytoplasm.

FUNCTION

Coenzyme A (CoA) functions as a carrier of acetyl and acyl groups in cells and thus plays an important role in numerous synthetic and degradative metabolic pathways in all organisms. In eukaryotes, CoA and its derivatives are also involved in membrane trafficking and signal transduction. This gene encodes the bifunctional protein coenzyme A synthase (CoAsy) which carries out the last two steps in the biosynthesis of CoA from pantothenic acid (vitamin B5). The phosphopantetheine adenylyltransferase domain of this bifunctional protein catalyzes the conversion of 4'-phosphopantetheine into dephospho-coenzyme A (dpCoA) while its dephospho-CoA kinase domain completes the final step by phosphorylating dpCoA to form CoA. Mutations in this gene are associated with neurodegeneration with brain iron accumulation (NBIA). Alternative splicing results in multiple isoforms. Bifunctional enzyme that catalyzes the fourth and fifth sequential steps of CoA biosynthetic pathway. The fourth reaction is catalyzed by the phosphopantetheine adenylyltransferase, coded by the coaD domain; the fifth reaction is catalyzed by the dephospho-CoA kinase, coded by the coaE domain. May act as a point of CoA biosynthesis regulation.