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FH Antibody (N-term)

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
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  • 1 - FH Antibody (N-term) AW5544
    All lanes : Anti-FH Antibody (N-term) at 1:1000 dilution Lane 1: Hela whole cell lysate Lane 2: 293T whole cell lysate Lane 3: NIH/3T3 whole cell lysate Lane 4: A431 whole cell lysate Lane 5: Jurkat whole cell lysate Lane 6: HepG2 whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 55 kDa Blocking/Dilution buffer: 5% NFDM/TBST.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB
Primary Accession P07954
Reactivity Human, Mouse
Host Rabbit
Clonality Polyclonal
Calculated MW H=55,50;M=54,50;R=54,50 KDa
Isotype Rabbit Ig
Antigen Source HUMAN
Additional Information
Gene ID 2271
Antigen Region 107-135
Other Names Fumarate hydratase, mitochondrial, Fumarase, FH
Dilution WB~~1:1000
Target/Specificity This FH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 107-135 amino acids from the N-terminal region of human FH.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsFH Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name FH
Function Also acts as a tumor suppressor.
Cellular Location Isoform Mitochondrial: Mitochondrion.
Tissue Location Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non- spherocytic hemolytic anemia of unknown etiology
Research Areas

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.

REFERENCES

Shimada, M., et al. Hum. Genet. 128(4):433-441(2010)
Allegri, G., et al. J. Inherit. Metab. Dis. 33(4):411-419(2010)
Yogev, O., et al. PLoS Biol. 8 (3), E1000328 (2010) :
Yang, Y., et al. Cancer Genet. Cytogenet. 196(1):45-55(2010)
Rikova, K., et al. Cell 131(6):1190-1203(2007)

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