Anti-FH Antibody Picoband® Fluoro488 Conjugated
Product Specifications
Background
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.
Synonyms
Fumarate hydratase, mitochondrial; Fumarase; 4.2.1.2; FH
Gene Name
FH
Gene ID
2271
UniProt
P07954
Host
Rabbit
Reactivity
Human, Mouse, Rat
Cross Reactivity
No cross-reactivity with other proteins.
Immunogen
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.
Clonality
Polyclonal
Tissue Specificity
Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non- spherocytic hemolytic anemia of unknown etiology.
Applications
Flow Cytometry
Field of Research
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
Purification
Immunogen affinity purified.
Form
Liquid
Function
Also acts as a tumor suppressor.
References & Citations
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.
Storage Conditions
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
Specificity
No cross reactivity with other proteins.
Applications Notes
6
Gene Name Synonym
Fumarate hydratase
Subcellular Location
Isoform Mitochondrial: Mitochondrion.
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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