Overview

  • Product name

    Anti-FH/Fumarase antibody (Biotin)
    See all FH/Fumarase primary antibodies
  • Description

    Goat polyclonal to FH/Fumarase (Biotin)
  • Host species

    Goat
  • Conjugation

    Biotin
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Pig, Zebrafish, Cynomolgus monkey
  • Immunogen

    Synthetic peptide corresponding to Human FH/Fumarase aa 176-189 (internal sequence) (Cysteine residue). (NP_000134.2)
    Sequence:

    C-HPNDHVNKSQSSND


    Database link: P07954

  • Positive control

    • WB: Human kidney lysate.
  • General notes

     This product was previously labelled as FH

     

Properties

Applications

Our Abpromise guarantee covers the use of ab239745 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB Use a concentration of 0.1 - 0.3 µg/ml. Predicted molecular weight: 55 kDa.

Target

  • Function

    Also acts as a tumor suppressor.
  • Pathway

    Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
  • Involvement in disease

    Defects in FH are the cause of fumarase deficiency (FHD) [MIM:606812]; also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.
    Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).
    Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].
  • Sequence similarities

    Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
  • Cellular localization

    Cytoplasm and Mitochondrion.
  • Information by UniProt
  • Database links

  • Alternative names

    • FH antibody
    • Fumarase antibody
    • Fumarate hydratase antibody
    • Fumarate hydratase mitochondrial antibody
    • Fumarate hydratase, mitochondrial antibody
    • FUMH_HUMAN antibody
    • HLRCC antibody
    • LRCC antibody
    • MCL antibody
    • MCUL 1 antibody
    • MCUL1 antibody
    • MS709 antibody
    • Multiple hereditary cutaneous leiomyomata antibody
    see all

Images

  • Anti-FH/Fumarase antibody (Biotin) (ab239745) at 0.1 µg/ml + Human kidney lysate (in RIPA buffer) at 35 µg

    Predicted band size: 55 kDa



    Primary incubation for 1 hour. Detected by chemiluminescence, using streptavidin-HRP and using NAP blocker as a substitute for skimmed milk.

References

ab239745 has not yet been referenced specifically in any publications.

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