| Overview |
| bsm-33167M-BF750 |
| Fumarase/FH (4C12) Monoclonal Antibody, AbBy Fluor-750 Conjugated |
| WB, IF |
| Human, Mouse, Rat |
| Specifications |
| AbBy Fluor-750 |
| Mouse |
| Recombinant human Fumarase/FH Protein |
| Monoclonal |
| #REF! |
| IgG |
| 1ug/ul |
| Purified by Protein G. |
| Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target |
| 2271 |
| P07954 |
| Cytoplasm, Mitochondrion matrix |
| FH; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata. |
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. [provided by RefSeq, Jul 2008]
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| Application Dilution |
| WB |
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| IF |
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