Rabbit anti-Human COASY Polyclonal Antibody

Uniprot Id

Q13057

Target Species

Human

Target Name

COASY

Target Full Name

Bifunctional coenzyme A synthase

Target Function

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.

Target Involvement

Neurodegeneration with brain iron accumulation 6 (NBIA6)

Target Subcellular Location

Cytoplasm. Mitochondrion matrix.

Target Protein Families

Eukaryotic CoaD family

Target Tissue Specificity

Expressed in all tissues examined including brain, heart, skeletal muscle, colon, thymus, spleen, kidney, liver, small intestine, placenta, lung and peripheral blood leukocyte. Lowest expression in peripheral blood leukocytes and highest in kidney and liv

Target Synonyms

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

Target Background

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.