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FH Antibody (N-term) (Ascites)

Mouse Monoclonal Antibody (Mab)

     
  • WB - FH Antibody (N-term) (Ascites) AM2120a
    FH Antibody (N-term)(Ascites)(Cat. #AM2120a) western blot analysis in Jurkat cell line lysates (35μg/lane).This demonstrates the FH antibody detected the FH protein (arrow).
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immuno electron microscopy
  • EIA=Enzyme Immunoassay
WB, E
Primary Accession P07954
Other Accession P14408, P10173, Q60HF9, NP_000134.2
Reactivity Human
Predicted Monkey, Pig, Rat
Host Mouse
Clonality Monoclonal
Isotype IgM
Clone/Animal Names 616CT6.1.6
Calculated MW 54637 Da
Antigen Region 35-63 aa
Additional Information
Gene ID 2271
Other Names Fumarate hydratase, mitochondrial, Fumarase, FH
Target/Specificity This FH antibody is generated from mice immunized with a KLH conjugated synthetic peptide between 35-63 amino acids from the N-terminal region of human FH.
Dilution WB~~1:100~1600
Format Mouse monoclonal antibody supplied in crude ascites with 0.09% (W/V) sodium azide.
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) (Ascites) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name FH {ECO:0000303|PubMed:27037871, ECO:0000312|HGNC:HGNC:3700}
Function Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed:30761759). Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable).
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
Citations (0)
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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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$ 182.50
Cat# AM2120a
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