Anti-Fumarase/FH antibody (44-510aa) [AT3F6] (STJA0041743)
SPECIFICATIONS
ClonalityMonoclonal
HostMouse
ConjugationUnconjugated
IsotypeIgG2ak
ImmunogenRecombinant human Fumarase (44-510aa) purified from E. coli
General Information
| Short Description | Mouse monoclonal anti-Fumarase/FH (44-510aa) for use in ELISA, WB, ICC, IF and FACS in Human samples. Datasheet included with dilution recommendations, and related reagents. |
| Applications | ELISA/WB/ICC/IF/FACS |
| Host | Mouse |
| Reactivity | Human |
| Note | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
Product Properties
| Clonality | Monoclonal |
| Clone ID | AT3F6 |
| Isotype | IgG2ak |
| Conjugation | Unconjugated |
| Concentration | 1 mg/mL |
| Purification | By protein-A affinity chromatography |
| Formulation | Liquid in phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% Glycerol |
| Storage Instruction | For short term storage, keep at +2C to +8C for up to 1 week. For long term storage, aliquot and store at-20C, and avoid repeat freeze-thaw cycles. |
Target Information
| Gene Symbol | FH |
| Gene ID | 2271 |
| Uniprot ID | FUMH_HUMAN |
| Accession Number | NP_000134 |
| Immunogen | Recombinant human Fumarase (44-510aa) purified from E. coli |
| Immunogen Region | 44-510aa |
Additional Info
| Tissue Specificity | Expressed in red blood cells.underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology. |
| Post Translational Modifications | Isoform Cytoplasmic: Phosphorylation at Thr-236 by PRKDC in response to DNA damage promotes translocation to the nucleus and recruitment to DNA double-strand breaks (DSBs). |
| Function | Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable). Isoform Mitochondrial: Catalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH. Isoform Cytoplasmic: Catalyzes the dehydration of L-malate to fumarate. Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism.fumarate being a by-product of the urea cycle and amino-acid catabolism. Also plays a role in DNA repair by promoting non-homologous end-joining (NHEJ). In response to DNA damage and phosphorylation by PRKDC, translocates to the nucleus and accumulates at DNA double-strand breaks (DSBs): acts by catalyzing formation of fumarate, an inhibitor of KDM2B histone demethylase activity, resulting in enhanced dimethylation of histone H3 'Lys-36' (H3K36me2). |
| Protein Name | Fumarate Hydratase - MitochondrialFumaraseHsfh |
| Database Links | Reactome: R-HSA-71403Reactome: R-HSA-9837999 |
| Cellular Localisation | Isoform Mitochondrial: MitochondrionIsoform Cytoplasmic: CytoplasmCytosolNucleusChromosomeTranslocates To The Nucleus In Response To Dna Damage: Localizes To Dna Double-Strand Breaks (Dsbs) Following Phosphorylation By Prkdc |
| Alternative Antibody Names | Anti-Fumarate Hydratase - Mitochondrial antibodyAnti-Fumarase antibodyAnti-Hsfh antibodyAnti-FH antibody |
Information sourced from Uniprot.org