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| Reactivity: | Human |
| Applications: | WB, ELISA |
| Host Species: | Rabbit |
| Isotype: | IgG |
| Clonality: | Polyclonal antibody |
| Gene Name: | fumarate hydratase |
| Gene Symbol: | FH |
| Synonyms: | MCL; FMRD; HsFH; LRCC; HLRCC; MCUL1; FH |
| Gene ID: | 2271 |
| UniProt ID: | P07954 |
| Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 50-150 of human FH (NP_000134.2). |
| Dilution: | WB 1:500-1:1000 |
| Purification Method: | Affinity purification |
| Concentration: | 0.49 mg/mL |
| Buffer: | PBS with 0.02% sodium azide, 50% glycerol ,pH7.3. |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. |
| Documents: | Manual-FH polyclonal antibody |
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.
Images
 | Western blot analysis of various lysates, using FH Rabbit pAb (A21403) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 10s. |
 | Western blot analysis of lysates from wild type(WT) and FH knockout (KO) 293T(KO) cells, using [KO Validated] FH Rabbit pAb (A21403) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s. |
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