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Fumarase Rabbit polyclonal Antibody IgG

SKU: BA113144-100µl
$279.00Price

Fig1: Western blot analysis of Fumarase on different lysates. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (ER1901-10, 1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1:5,000 dilution was used for 1 hour at room temperature.

Positive control: 

Lane 1: K562 cell lysate

Lane 2: Mouse colon tissue lysate

Fig2: ICC staining of Fumarase in LoVo cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 1% Blocker BSA for 15 minutes at room temperature. Cells were probed with the primary antibody (ER1901-10, 1/200) for 1 hour at room temperature, washed with PBS. Alexa Fluor®488 Goat anti-Rabbit IgG was used as the secondary antibody at 1/100 dilution. The nuclear counter stain is DAPI (blue).

Fig3: ICC staining of Fumarase in SH-SY5Y cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 1% Blocker BSA for 15 minutes at room temperature. Cells were probed with the primary antibody (ER1901-10, 1/50) for 1 hour at room temperature, washed with PBS. Alexa Fluor®488 Goat anti-Rabbit IgG was used as the secondary antibody at 1/100 dilution. The nuclear counter stain is DAPI (blue).

Bon Opus Cat. #BA113144
Size
  • Host Species; Species Reactivity

    Rabbit; Human, Mouse, Rat
  • Immunogen

    Recombinant protein within human Fumarase aa 83-267.
  • Application Summary

    WB, ICC, IHC
  • Purification; Formulation

    Protein affinity purified.; 1*TBS (pH7.4), 1%BSA, 50%Glycerol. Preservative: 0.05% Sodium Azide.
  • 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. While homozygous mutations of FH leads to fumaric aciduria, a lethal metabolic disorder, its heterozygous mutations cause hereditary leiomyomatosis and renal cell cancer (HLRCC), a cancer syndrome characterized by uterine fibroids, cutaneous leiomyoma, and type 2 papillary renal cell cancer. The oncogenic properties of FH loss have been mostly ascribed to the intracellular accumulation of fumarate.(ER1901-10)