Coenzyme A synthase (COASY)

Coenzyme A synthase is a bifunctional enzyme encoded by the COASY gene in humans. It catalyzes the final two steps in the biosynthesis of coenzyme A from pantothenic acid (vitamin B5): first converting 4'-phosphopantetheine to dephospho-coenzyme A via its phosphopantetheine adenylyltransferase domain, then phosphorylating dephospho-coA to form coenzyme A via its dephospho-CoA kinase domain. This enzyme is essential for cellular metabolism as coenzyme A acts as a carrier of acetyl and acyl groups in numerous metabolic pathways including fatty acid synthesis and oxidation, energy production through the citric acid cycle, and regulation of gene expression. Mutations in COASY are associated with rare neurodegenerative diseases characterized by brain iron accumulation. The enzyme is primarily localized to mitochondria but can also be found in cytoplasmic and nuclear compartments.

Other names

Bifunctional coenzyme A synthaseCoAsyCOASY protein