- Clone
- 9B1-4E1-8D5 (See other available formats)
- Regulatory Status
- RUO
- Other Names
- Fumarate Hydratase, FH, MCL, HLRCC, MCUL1, FMRD
- Isotype
- Mouse IgG1, κ
- Ave. Rating
- Submit a Review
- Product Citations
- publications
Cat # | Size | Price | Quantity Check Availability | Save | ||
---|---|---|---|---|---|---|
616851 | 25 µg | 134€ | ||||
616852 | 100 µg | 333€ |
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.
Product DetailsProduct Details
- 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)
Antigen Details
- 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 View all products for this Gene ID
- UniProt
- View information about Fumarase on UniProt.org
Related Pages & Pathways
Pages
Other Formats
View All Fumarase Reagents Request Custom ConjugationDescription | Clone | Applications |
---|---|---|
Purified anti-Fumarase | 9B1-4E1-8D5 | WB,IHC-P,ICC |
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