| CatalogCode: | NB100-61663 |
| ProductName: | Fumarase Antibody |
| Product Description: | Goat Polyclonal anti-Fumarase / FH |
| Clonality: | Polyclonal |
| Immunogen: | Synthetic peptide, C-HPNDHVNKSQSSND, representing the internal region of the sequence according to NP_000134.2 |
| Epitope: | HPNDHVNKSQSSND |
| CrossReactivity: | Human. Expected based upon sequence homology: Human, Mouse, Rat, Dog. |
| Packaging: | 0.1 mg Immunogen affinity purified Goat antisera. |
| Uses: | Peptide ELISA: antibody detection limit dilution 1:64,000. Approx 48kDa band observed in Human Kidney, Liver and Lung lysates (calculated MW of 54.6kDa according to NP_000134.2). The observed molecular weight corresponds to earlier findings with antibodies from different sources. Recommended concentration: 0.01-0.03ug/ml. |
| Control: | Human Kidney lysate |
| Background: | The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. |
| Storage: | Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles. |
| Purity: | Immunogen affinity purified |
| Isotype: | IgG |
| Host_Name: | Goat |
| Buffer: | Tris-saline [pH7.3] with 0.5% BSA |
| ListPrice: | 295 |
| AppSummary: | ELISA, WB |
| SpeciesSummary: | Hu |
| ALTnames: | anti-fumarate hydratase antibody, anti-HLRCC antibody, anti-LRCC antibody, anti-MCL antibody, anti-MCUL1 antibody, anti-fumarase antibody, anti-leiomyomatosis and renal cell cancer antibody, anti-multiple hereditary cutaneous leiomyomata antibody |
| ProteinTarget: | FH |
| PackageSize: | 0.1 mg |
| Homology: | Human, Mouse, Rat, Dog |
| GeneralRef: | Pollard PJ, Spencer-Dene B, Shukla D, Howarth K, Nye E, El-Bahrawy M, Deheragoda M, Joannou M, McDonald S, Martin A, Igarashi P, Varsani-Brown S, Rosewell I, Poulsom R, Maxwell P, Stamp GW, Tomlinson IP. Targeted inactivation of fh1 causes proliferative renal cyst development and activation of the hypoxia pathway. Cancer Cell. 2007 Apr;11(4):311-9. PMID: 17418408 |
| NotesMain: | GeneIDs all Nonhuman: 14194 (mouse); 24368 (rat); |
| Gene_Id: | 2271 |
| Ref_Sqnce: | NP_000134.2 |
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