Recombinant
RabMAb

Recombinant Anti-Frataxin antibody [EPR6107] - BSA and Azide free (ab244232)

Overview

  • Product name

    Anti-Frataxin antibody [EPR6107] - BSA and Azide free
    See all Frataxin primary antibodies
  • Description

    Rabbit monoclonal [EPR6107] to Frataxin - BSA and Azide free
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
    Unsuitable for: IHC-P
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide within Human Frataxin aa 150 to the C-terminus. The exact sequence is proprietary.

  • General notes

    ab244232 is the carrier-free version of ab124680 This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.

     

    Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.

    Use our conjugation kits  for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.

    Ab244232 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.

    Maxpar® is a trademark of Fluidigm Canada Inc.

    Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab244232 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 at an assay dependent concentration. Detects a band of approximately 17, 30 kDa (predicted molecular weight: 19, 23 kDa).
  • Application notes
    Is unsuitable for IHC-P.
  • Target

    • Function

      Promotes 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 specificity

      Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
    • Involvement in disease

      Defects 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 similarities

      Belongs to the frataxin family.
    • Post-translational
      modifications

      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 localization

      Cytoplasm. 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

    References

    ab244232 has not yet been referenced specifically in any publications.

    Customer reviews and Q&As

    There are currently no Customer reviews or Questions for ab244232.
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    Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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