Ketone bodies are synthesized in the liver from fatty acids and are utilized by extrahepatic tissues as an energy source when glucose levels are low, such as during fasting or carbohydrate restriction. The three main ketone bodies are acetoacetate, beta-hydroxybutyrate, and acetone. Ketogenesis is regulated by the mobilization of fatty acids from adipose tissue and the partitioning of acetyl-CoA between the ketogenesis pathway and the citric acid cycle in the liver. Elevated levels of ketone bodies in the blood (ketonemia) or urine (ketonuria), known as ketosis, can occur during starvation, prolonged exercise, or in pathological states like uncontrolled diabetes mellitus.