Coenzyme A (CoA) plays a crucial role in energy metabolism and is implicated in a large number of disorders, from ultra-rare diseases like pantothenate-kinase associated neurodegeneration, or PKAN, and organic acidurias (OAs), to common diseases such as Type 2 diabetes.
CoA and its related derivatives, which are called thioesters, are used by approximately 4% of cellular enzymes. CoA thioesters regulate the crossroads of energy metabolism. They are involved in some of the most important pathways responsible for breaking down metabolites and building key molecules necessary for healthy physiology. These pathways include the Krebs cycle, fatty acid synthesis and degradation, ketogenesis and steroidogenesis.