Description_x000D_ General description_x000D_ Fumarase (FH in human) is a well-known tricarboxylic-acid-cycle enzyme found in both the cytoplasm and mitochondria of all eukaryotes._x000D_ Fumarase is mapped to human chromosome 1q43. Fumarase comprises of three domains D1, D2 and D3 and belongs to class II fumarase family._x000D_ Application_x000D_ Fumarase from porcine heart has been used:_x000D_ • as a positive control in isocitrate dehydrogenase and α-ketoglutarate dehydrogenase assay_x000D_ • for immobilized enzyme based stability assay_x000D_ • in fumarate hydratase assays in human lung carcinoma cells (A549)_x000D_ Fumarase is used as a protein calibration standard in the purification of intact DNA polymerase a/primase from mouse cells._x000D_ Biochem/physiol Actions_x000D_ Fumarase catalyzes the reversible hydration of fumarate to malate. In its mitochondrial form, fumarate is involved in the Krebs Cycle, while the cytosolic form is involved in amino acid metabolism._x000D_ Fumarase is essential for regulation of cellular metabolism. Fumarase elicits protective function from double-strand breaks (DSBs) induced by infrared radiation. Loss of function mutations in the fumarase gene is implicated in hereditary leiomyomatosis and renal cell cancer (HLRCC)-syndrome._x000D_ Unit Definition_x000D_ One unit will convert 1.0 μmole of L-malate to fumarate per min at pH 7.6 at 25 °C._x000D_ Physical form_x000D_ Suspension in 3.2 M (NH4)2SO4, 0.05 M KH2PO4, pH 7.5, 0.014 M 2-mercaptoethanol_x000D_ Preparation Note_x000D_ Isolated by the method of Beeckmans, S., and Kanarek, L., Eur. J. Biochem., 78, 437 (1977). Affinity purified.