Human Fumarase/FH protein (Recombinant) (No-Tag) (STJP019626)
SPECIFICATIONS
HostE.coli
ImmunogenHuman
General Information
| Short Description | Recombinant-Human Fumarase/FH-No-Tag protein was developed from e.coli and has a target region of No-Tag. For use in research applications. |
| Applications | SDS-PAGE/Enzyme Activity |
| Host | E.coli |
| Note | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
Product Properties
| Concentration | 1 mg/mL |
| Formulation | Liquid in 20mM Tris-HCl buffer (pH 8.0) |
| 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. |
| Endotoxin | < 1 EU per 1ug of protein (determined by LAL method) |
| Immunoreactivity | Specific activity is > 25 unit/mg, and is defined as the amount of enzyme that cleaves 1umole of L-Malate to Fumarate per minute at pH 7.5 at 37C. |
Target Information
| Gene Symbol | FH |
| Gene ID | 2271 |
| Uniprot ID | FUMH_HUMAN |
| Accession Number | NP_000134 |
| Immunogen | Human |
| Immunogen Region | 44-510aa |
| Immunogen Sequence | MASQNSFRIE YDTFGELKVP NDKYYGAQTV RSTMNFKIGG VTERMPTPVI KAFGILKRAA AEVNQDYGLD PKIANAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV NEVISNRAIE MLGGELGSKI PVHPNDHVNK SQSSNDTFPT AMHIAAAIEV HEVLLPGLQK LHDALDAKSK EFAQIIKIGR THTQDAVPLT LGQEFSGYVQ QVKYAMTRIK AAMPRIYE |
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 Protein Names | Fumarate Hydratase - Mitochondrial proteinFumarase proteinHsfh proteinFH protein |
Information sourced from Uniprot.org