• Product nameAnti-Frataxin antibody
    See all Frataxin primary antibodies
  • Description
    Mouse monoclonal to Frataxin
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Recombinant Fragment
    Predicted to work with: Human
  • Immunogen

    Recombinant fragment, corresponding to amino acids 91-201 of Human Frataxin



Our Abpromise guarantee covers the use of ab55021 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
  • Application notesWB: Use at a concentration of 1-5 µg/ml.

    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • Target

    • FunctionPromotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1.
    • Tissue specificityExpressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
    • Involvement in diseaseDefects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region.
    • Sequence similaritiesBelongs to the frataxin family.
    • Post-translational
      Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure.
    • Cellular localizationCytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria.
    • Information by UniProt
    • Database links
    • Alternative names
      • CyaY antibody
      • d-FXN antibody
      • FA antibody
      • FARR antibody
      • Frataxin mature form antibody
      • Frataxin(81-210) antibody
      • FRDA antibody
      • FRDA_HUMAN antibody
      • Friedreich ataxia protein antibody
      • Fxn antibody
      • i-FXN antibody
      • m56-FXN antibody
      • m78-FXN antibody
      • m81-FXN antibody
      • MGC57199 antibody
      • X25 antibody
      see all

    Anti-Frataxin antibody images

    • Western blot against tagged recombinant protein immunogen using ab55021 Frataxin antibody at 1ug/ml. Predicted band size of immunogen is 37 kDa.

      This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    References for Anti-Frataxin antibody (ab55021)

    ab55021 has not yet been referenced specifically in any publications.

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