Biolegend, 616851, Purified anti-Fumarase Antibody, 25μg

Fumarase, or fumarate hydratase, is a Krebs cycle enzyme that catalyzes the conversion of fumarate to L-malate. A cytosolic isoform of fumarase has been described that is required for arginine metabolism and the urea cycle. Inactivating germline mutations of the FH gene are found in patients with hereditary leiomyomatosis and renal cell cancer syndrome, which predisposes patients to increased risk of developing uterine, cutaneous leiomyomas, and renal cell carcinomas. Mechanistically, elevated fumarate functions as an oncometabolite by inhibiting the activity of α-ketoglutarate dependent enzymes, such as hypoxia-inducible factor prolyl hydroxylases (PHDs) and ten-eleven translocation (TET) dioxygenases.
25μg
Verified Reactivity: Human
Antibody Type: Monoclonal
Host Species: Mouse
Immunogen: Recombinant fragment of human fumarase
Formulation: Phosphate-buffered solution, pH 7.2, containing 0.09% sodium azide
Preparation: The antibody was purified by affinity chromatography.
Concentration: 0.5 mg/mL
Storage & Handling: The antibody solution should be stored undiluted between 2°C and 8°C.
Application: WB - Quality tested IHC-P, ICC - Verified
Recommended Usage: Each lot of this antibody is quality control tested by western blotting. For western blotting, the suggested use of this reagent is 0.25 - 1.0 µg/mL. For immunohistochemistry on formalin-fixed paraffin-embedded tissue sections, a concentration range of 1.0 - 10.0 µg/mL is suggested. For immunocytochemistry, a concentration range of 1.25 - 5.0 μg/mL is recommended. It is recommended that the reagent be titrated for optimal performance for each application.
Application Notes: This product does not recognize mouse and rat Fumarase. For cross-reactivity to mouse and rat, refer to clone W18319C (Cat. No. 940001). For immunocytochemistry, ice-cold methanol-only fixation is recommended.
RRID: AB_3068159 (BioLegend Cat. No. 616851) AB_3068159 (BioLegend Cat. No. 616852)
Structure: Fumarase is a 510 amino acid protein with a predicted molecular weight of 55 kD.
Distribution: Ubiquitously expressed/Mitochondrial, Cytosolic
Function: Catalyzes fumarate to L-malate
Interaction: H2AZ1
Biology Area: Cell Biology, Mitochondrial Function
Molecular Family: Enzymes and Regulators
Antigen References: Laukka T, et al. 2016. J Biol Chem. 291:4256-65. Sciavoceli M, et al. 2016. Nature. 537:544-547.
Gene ID: 2271
UniProt: View information about Fumarase on UniProt.org
Clone: 9B1-4E1-8D5
Regulatory Status: RUO
Other Names: Fumarate Hydratase, FH, MCL, HLRCC, MCUL1, FMRD
Isotype: Mouse IgG1, κ