The FH/Fumarase Rabbit mAb Antibody from MyBioSource.com is a Rabbit Monoclonal antibody to FH, and Fumarate Hydratase. This antibody recognizes Human, Mouse, and Rat antigen. The FH/Fumarase Rabbit mAb Antibody has been shown to work in the following applications: Flow Cytometry, Immunocytochemistry, Immunofluorescence, Immunohistochemistry, Immunoprecipitation, and Western Blot.
Description
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. [provided by RefSeq, Jul 2008]
Also acts as a tumor suppressor.MiscellaneousThere are 2 substrate-binding sites: the catalytic A site, and the non-catalytic B site that may play a role in the transfer of substrate or product between the active site and the solvent. Alternatively, the B site may bind allosteric effectors