The enzyme, succinyl coenzyme A synthetase, acts on the conversion of the succinyl group succinate. A decrease in the ATP production of the mitochondria may occur if there is a deficiency of this enzyme. The decrease is seen during the phosphorylation of succinyl CoA to succinate. With this decrease, the cells would produce lactic acid in order to get ATP leading the cell to be non-functional. The deficiency of the enzyme, and the increase in lactic acid causes fatal infantile lactic acid acidosis. In the tricarboxylic acid cycle or TCA cycle, there would also be a decrease of ATP in the mitochondria, a decrease in NADH, and the same carbon dioxide level. All of this decrease in ATP production is related to the deficiency of succinyl CoA synthetase.
