Purified anti-Fumarase Antibody, Fumarase, W18319C,BioLegend,940001

Fumarate hydratase, or fumarase, is a Krebs cycle enzyme that catalyzes the conversion of fumarate to L-malate. A cytosolic isoform of Fumarase has been described that is required for arginine metabolism and the urea cycle. Inactivating germline mutations of the FH gene are found in patients with hereditary leiomyomatosis and renal cell cancer syndrome, which predisposes patients to increased risk of developing uterine and cutaneous leipmyomas and renal cell carcinomas. Mechanistically, elevated fumarate functions as an oncometabolite by inhibiting the activity of α-ketoglutarate dependent enzymes, such as hypoxia-inducible factor prolyl hydroxylases (PHDs) and ten-eleven translocation (TET) dioxygenases.