CoA is important within the mitochondria because it plays an essential role in transporting fatty acids from the cell cytoplasm to the mitochondrial matrix as well as feeding into the beginning of the citric acid cycle. Being able to continuously recycle CoA and make additional Acyl-CoA is critical to allow for prolonged functioning of the mitochondria. CoA allows for the recycling of carnitine for the carnitine shuttle, which brings fatty acids into the mitochondrial matrix for beta oxidation.
Sequestration of CoA as a medium-chain acyl CoA ester can result from medium-chain acyl CoA dehydrogenase (MDAC) mutations and deficiencies. This inhibits the ability of the mitochondria to break down fatty acids into acetyl-CoA, greatly reducing the
