Fumarate Hydratase, Mitochondrial

E4337hu

Sandwich

48T,96T

9.88ng/L

20-4500ng/L

Serum, plasma, cell culture supernates

Human

2-8ºC

1h 30m

Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed: 30761759). Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable). [Isoform Mitochondrial]: Catalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH. [Isoform Cytoplasmic]: Catalyzes the dehydration of L-malate to fumarate (By similarity). Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism; fumarate being a by-product of the urea cycle and amino-acid catabolism (By similarity). Also plays a role in DNA repair by promoting non-homologous end-joining (NHEJ) (PubMed: 20231875, PubMed: 26237645). In response to DNA damage and phosphorylation by PRKDC, translocates to the nucleus and accumulates at DNA double-strand breaks (DSBs): acts by catalyzing formation of fumarate, an inhibitor of KDM2B histone demethylase activity, resulting in enhanced dimethylation of histone H3 'Lys-36' (H3K36me2) (PubMed: 26237645).

54,637

2271

FH;Fumarase;Fumarate hydratase, mitochondrial;HsFH

FH

Others

FH