| Host: |
Rabbit |
| Applications: |
WB/IHC/IF |
| Reactivity: |
Human/Mouse/Rat |
| Note: |
STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Short Description: |
Rabbit polyclonal antibody anti-FH (45-510) is suitable for use in Western Blot, Immunohistochemistry and Immunofluorescence research applications. |
| Clonality: |
Polyclonal |
| Conjugation: |
Unconjugated |
| Isotype: |
IgG |
| Formulation: |
PBS with 0.02% Sodium Azide, 50% Glycerol, pH7.3. |
| Purification: |
Affinity purification |
| Dilution Range: |
WB 1:100-1:500IHC-P 1:50-1:200IF/ICC 1:50-1:100 |
| Storage Instruction: |
Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
| Gene Symbol: |
FH |
| Gene ID: |
2271 |
| Uniprot ID: |
FUMH_HUMAN |
| Immunogen Region: |
45-510 |
| Immunogen: |
Recombinant fusion protein containing a sequence corresponding to amino acids 45-510 of human FH (NP_000134.2). |
| Immunogen Sequence: |
ASQNSFRIEYDTFGELKVPN DKYYGAQTVRSTMNFKIGGV TERMPTPVIKAFGILKRAAA EVNQDYGLDPKIANAIMKAA DEVAEGKLNDHFPLVVWQTG SGTQTNMNVNEVISNRAIEM LGGELGSKIPVHPNDHVNKS QSSNDTFPTAMHIAAAIEVH EVLLPGLQKLHDALDAKSKE FAQIIKIGRTHTQDAVPLTL GQEFSGYVQQVKYAMTRIKA AMPRIYELAAGGTAVGTGL |
| 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-71403 |
| 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
