Fumarase


UniProt Number: P07954
Alternate Names: FH, fumarate hydratase, L-malate hydrolyase, S-malate hydrolyase
Structure and Function: Fumarase catalyzes the reversible hydration/dehydration of fumarate to S-malate. Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial form plays a role in the Citric Acid cycle and the cytosolic form is involved in the metabolism of amino acids and fumarate. Subcellular localization is established by the presence of a signal sequence on the amino terminus in the mitochondrial form.
Disease Associations: Defects in FH are the cause of fumarase deficiency (FD) [MIM:606812] (also known as fumaricaciduria), multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800], and hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].


Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount
MS709 Fumarase antibody human, bovine, rat ICC, IHC, IP, ICE, FLOW 100 µg


Enzyme Activity Assays
Cat. No. Name Reactivity Amount
MS780 Fumarase Specific Activity Assay Kit human, rat (activity only) 96 tests


Protein Quantity Assays
Cat. No. Name Reactivity Amount 
ab157399 Fumarase Human ELISA profiling Kit human 96 tests



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