COASY Antibody – Mouse Monoclonal

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. [provided by RefSeq, Apr 2014]

PPAT: bifunctional cytoplasmic 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.

Protein type: EC 2.7.7.3; EC 2.7.1.24; Transferase; Kinase, other; Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis

Chromosomal Location of Human Ortholog: 17q12-q21

Cellular Component: nucleoplasm; mitochondrial outer membrane; mitochondrial matrix; cytoplasm

Molecular Function: protein binding; dephospho-CoA kinase activity; pantetheine-phosphate adenylyltransferase activity; ATP binding

Biological Process: coenzyme biosynthetic process; coenzyme A biosynthetic process; vitamin metabolic process; pantothenate metabolic process; phosphorylation; water-soluble vitamin metabolic process

Disease: Neurodegeneration With Brain Iron Accumulation 6