FH-/- CL 1 Cell Line
Fumarate hydratase – which can be found in either a mitochondrial or cytosolic form – is an enzyme that catalyzes the reversible hydration and dehydration of fumarate to malate. Signal sequences located in the protein dictate the subcellular location of each FH isoenzyme. The cytosolic form is involved in the metabolism of amino acids and fumarate and the mitochondrial form is involved in the Krebs Cycle (tricarboxylic acid cycle or the citric acid cycle). Germline mutations of FH are responsible for hereditary leiomyomatosis and renal-cell cancer (HLRCC)1.
