CPT1A, or Carnitine Palmitoyltransferase 1A, is an enzyme located in the mitochondria of liver cells. It plays a crucial role in the metabolism of fatty acids by facilitating the transport of long-chain fatty acids into the mitochondria, where they are broken down for energy. This process is essential for maintaining energy levels, especially during fasting or prolonged exercise. Mutations in the CPT1A gene can lead to a condition known as CPT1A deficiency, which affects the body's ability to metabolize certain fats. Individuals with this deficiency may experience symptoms such as hypoketotic hypoglycemia, especially during periods of fasting. Early diagnosis and management are important for preventing complications associated with this metabolic disorder.