Anti-FH Antibody Picoband® Fluoro647 Conjugated

Fumarase (or fumaratehydratase) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate. Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial isoenzyme is involved in the Krebs Cycle (also known as the Tricarboxylic Acid Cycle [TCA] or the Citric Acid Cycle), and the cytosolic isoenzyme is involved in the metabolism of amino acids and fumarate. Subcellular localization is established by the presence of a signal sequence on the amino terminus in the mitochondrial form, while subcellular localization in the cytosolic form is established by the absence of the signal sequence found in the mitochondrial variety. This enzyme participates in 2 metabolic pathways: citric acid cycle, reductive citric acid cycle (CO2 fixation), and is also important in renal cell carcinoma. Mutations in this gene have been associated with the development of leiomyomas in the skin and uterus in combination with renal cell carcinoma.

A synthetic peptide corresponding to a sequence at the N-terminus of human FH, which shares 100% and 97.8% amino acid (aa) sequence identity with mouse and rat FH, respectively.

Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non- spherocytic hemolytic anemia of unknown etiology.

Cancer, Cardiovascular, Fatty Acid Oxidation, Fatty Acids, Lipid and Lipoprotein Metabolism, Lipid Metabolism, Lipids/Lipoproteins, Metabolic Signaling Pathways, Metabolism, Pathways and Processes, Redox Metabolism, Signal Transduction

1. Begley TP, McMurry J (2005) . The organic chemistry of biological pathways. Roberts and Co. Publishers. 2. Weaver T (October 2005) . Structure of free fumarase C from Escherichia coli. ActaCrystallogr. D. 61 (Pt 10) : 1395–401.