Anti-FH antibody (50-150) (STJ11104324)
SPECIFICATIONS
ClonalityPolyclonal
HostRabbit
ConjugationUnconjugated
IsotypeIgG
General Information
| Short Description | Rabbit polyclonal Fumarate Hydratase-Mitochondrial (50-150) antibody for use in WB and ELISA in human, mouse and rat samples. Datasheet included with dilution recommendations, and related reagents. |
| Applications | WB/ELISA |
| Host | Rabbit |
| Reactivity | Human/Mouse/Rat |
| Note | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
Product Properties
| Clonality | Polyclonal |
| Isotype | IgG |
| Conjugation | Unconjugated |
| Concentration | Lot specific |
| Purification | Affinity purification |
| Dilution Range | WB:1:500-1:1000ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Formulation | PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. |
| Storage Instruction | Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
Target Information
| Gene Symbol | FH |
| Gene ID | 2271 |
| Uniprot ID | FUMH_HUMAN |
| Immunogen Region | 50-150 |
| Immunogen Sequence | FRIEYDTFGELKVPNDKYYG AQTVRSTMNFKIGGVTERMP TPVIKAFGILKRAAAEVNQD YGLDPKIANAIMKAADEVAE GKLNDHFPLVVWQTGSGTQT N |
| Specificity | A synthetic peptide corresponding to a sequence within amino acids 50-150 of human FH (NP_000134.2). |
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