FH Monoclonal Antibody
SKU: E-AB-22031-200
FH Monoclonal Antibody
| SKU # | E-AB-22031 |
| Reactivity | Human, Mouse, Rat |
| Host | Mouse |
| Applications | WB, IHC-p, IF |
Product Details
| Isotype | IgG |
| Host | Mouse |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC-p, IF |
| Clonality | Monoclonal |
| Immunogen | Synthetic Peptide |
| Abbre | FH |
| Synonyms | FH, FUMH, Fumarase, Fumarate hydratase, Fumarate hydratase mitochondrial, HLRCC, LRCC, MCL, MCUL 1, MCUL1, MS709, Multiple hereditary cutaneous leiomyomata, mitochondrial |
| Swissprot | |
| Observed MW | 50 kDa |
| Cellular Localization | Cytoplasm and Mitochondrion. |
| Tissue Specificity | Expressed in red blood cells, underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology. |
| Concentration | 1 mg/mL |
| Buffer | Phosphate buffered solution, pH 7.4, containing 0.05% stabilizer, 0.5% protein protectant and 50% glycerol. |
| Purification Method | Protein A purification |
| Research Areas | Cancer, Metabolism, Signal Transduction |
| Clone No. | 5H2 |
| Conjugation | Unconjugated |
| Storage | Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles. |
| Shipping | The product is shipped with ice pack,upon receipt,store it immediately at the temperature recommended. |
Related Reagents
| Applications | Recommended Dilution |
| WB | 1:500-1:3000 |
| IHC | 1:50-300 |
| IF | 1:100-1:300 |
Background
The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.