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Goat Anti-Fumarase / FH Antibody

Peptide-affinity purified goat antibody

     
  • EIA - Goat Anti-Fumarase / FH Antibody AF1449a
    EB7874 (1.5ug/ml) as the reporter with EB002023 as the capture rabbit antibody (2ug/ml).
    detail
  • WB - Goat Anti-Fumarase / FH Antibody AF1449a
    AF1449a (0.01µg/ml) staining of Human Kidney lysate (35µg protein in RIPA buffer). Detected by chemiluminescence.
    detail
  • IHC - Goat Anti-Fumarase / FH Antibody AF1449a
    AF1449a (3.75µg/ml) staining of paraffin embedded Human Skeletal Muscle. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.
    detail
  • IHC - Goat Anti-Fumarase / FH Antibody AF1449a
    AF1449a (3.75µg/ml) staining of paraffin embedded Human Heart. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.
    detail
  • SPECIFICATION
  • CITATIONS
  • PROTOCOLS
  • BACKGROUND
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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, IHC, Pep-ELISA
Primary Accession P07954
Other Accession NP_000134, 2271, 14194 (mouse), 24368 (rat)
Reactivity Human
Predicted Mouse, Rat, Dog
Host Goat
Clonality Polyclonal
Concentration 100ug/200ul
Isotype IgG
Calculated MW 54637 Da
Additional Information
Gene ID 2271
Other Names Fumarate hydratase, mitochondrial, Fumarase, 4.2.1.2, FH
Format 0.5 mg IgG/ml in Tris saline (20mM Tris pH7.3, 150mM NaCl), 0.02% sodium azide, with 0.5% bovine serum albumin
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsGoat Anti-Fumarase / FH Antibody 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)
citation

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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

An approach based on a genome-wide association study reveals candidate loci for narcolepsy. Shimada M, et al. Hum Genet, 2010 Oct. PMID 20677014.
Fumarase: a mitochondrial metabolic enzyme and a cytosolic/nuclear component of the DNA damage response. Yogev O, et al. PLoS Biol, 2010 Mar 9. PMID 20231875.
UOK 262 cell line, fumarate hydratase deficient (FH-/FH-) hereditary leiomyomatosis renal cell carcinoma: in vitro and in vivo model of an aberrant energy metabolic pathway in human cancer. Yang Y, et al. Cancer Genet Cytogenet, 2010 Jan 1. PMID 19963135.
Follow-up examination of linkage and association to chromosome 1q43 in multiple sclerosis. McCauley JL, et al. Genes Immun, 2009 Oct. PMID 19626040.
Novel role of fumarate metabolism in dahl-salt sensitive hypertension. Tian Z, et al. Hypertension, 2009 Aug. PMID 19546378.

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$ 341.00
Cat# AF1449a
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Availability: 7-10 days
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