| Host: |
Rabbit |
| Applications: |
WB/ICC/IF |
| Reactivity: |
Human/Mouse/Rat/Hamster |
| Note: |
STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Short Description: |
Rabbit monoclonal antibody anti-FH (45-59 aa) is suitable for use in Western Blot, Immunocytochemistry and Immunofluorescence research applications. |
| Clonality: |
Monoclonal |
| Clone ID: |
R08-3F2 |
| Conjugation: |
Unconjugated |
| Isotype: |
IgG |
| Formulation: |
50mM Tris-Glycine (pH7.4) , 0.15M NaCl, 40% Glycerol, 0.01% Sodium azide and 0.05% BSA |
| Purification: |
Affinity Purified |
| Concentration: |
0.3 mg/ml |
| Dilution Range: |
WB 1:500-1:1000IF 1:50-1:200 |
| Storage Instruction: |
Store at 4°C short term. Aliquot and store at-20°C long term. Avoid freeze/thaw cycles. |
| Gene Symbol: |
FH |
| Gene ID: |
2271 |
| Uniprot ID: |
FUMH_HUMAN |
| Immunogen Region: |
45-59 aa |
| Immunogen: |
A synthetic peptide of human FH |
| 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
12 months for antibodies. 6 months for ELISA Kits. Please see website T&Cs for further guidance
