Fumarate Hydratase, Mitochondrial
The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) 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.
Mouse Anti-FH Antibody (CBMOAB-33245FYC) (CAT#: CBMOAB-33245FYC)
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- Host species: Mouse
- Species Reactivity: A. thaliana (Arabidopsis thaliana), Cattle (Bos taurus), Chimpanzee (Pan troglodytes), Human (Homo sapiens), Mouse (Mus musculus), Rat (Rattus norvegicus), Pig (Sus scrofa), Chicken (Gallus gallus), Primate, Sheep (Ovis aries), Zebrafish (Danio rerio), Marmoset, Rhesus (Macaca mulatta), Zebrafish
- Application: WB, ELISA
- Protein: Frataxin, mitochondrial
- Size: 0.5mg, 1mg, 200µg
- Conjugate: Alexa Fluor 350, AP, APC, Biotin, Consult us more, Cy3, Cy5, Cy5.5, Cy7, FITC, HRP, None, PE, PerCP
- Alternative Names: Fumarate Hydratase, EC 4.2.1.2, Fumarase, Fumarate Hydratase, Mitochondrial, HLRCC, MCUL1, FMRD, LRCC, MCL
For Research Use Only | Not For Clinical Use.
