Fumarate hydratase is an enzyme that plays a crucial role in the Krebs cycle, which is essential for cellular respiration. It catalyzes the reversible hydration of fumarate to produce malate, facilitating the conversion of nutrients into energy. This process occurs in the mitochondria of cells. Mutations in the gene encoding fumarate hydratase can lead to fumarate hydratase deficiency, a rare genetic disorder. This condition is associated with various health issues, including hereditary leiomyomatosis and renal cell cancer. Understanding the function of fumarate hydratase is important for developing potential treatments for related diseases.