Name: Mouse anti-human Fumarate Hydratase Antibody (Clon J-13)
Description and aplications: The fumarate hydratase protein (FH) is an enzyme of the Krebs cycle that catalyzes the reversible and sterospecific transformation of the fumarate to Fmalate.There exist two isoforms, the FH1 located a cytosolic level, where is responsible for the hydratio from the fumarate to L-malate, and the mitochondrial FH2, responsible for the dehydration from the Lmalate to fumarate. The secretion of the enzyme is coded by a gene located in the chromosome region 1q43. The lack of this gene determines severe metabolic disorders characterized by early hypotonia, psychomotor retardation and brain anomalies such as agenesis of the corpus callosus, anomalies in the gyri and ventriculomegaly.
Composition: Composition: anti-human Fumarate Hydratase mouse monoclonal antibody purified from serum and prepared in 10mM PBS, pH 7.4, with 0.2% BSA and 0.09% sodium azide
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