This Antibody was verified by Knockdown to ensure that the antibody binds to the antigen stated. View Details
Recommended positive controls: 293T, A431, HeLa, HepG2, mouse liver, rat liver.
Predicted reactivity: Rat (98%), Zebrafish (90%), Pig (98%), Chicken (91%), Sheep (99%), Chimpanzee (99%), Bovine (98%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
The protein encoded by this gene is an enzymatic component of the tricarboxylic acid cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.
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Protein Aliases: EF-3; Fumarase; fumarate hydratase 1; Fumarate hydratase, mitochondrial
Gene Aliases: FH; Fh-1; Fh1; FMRD; HLRCC; LRCC; MCL; MCUL1
Molecular Function: lyase