Anti-Frataxin antibody [18A5DB1] (Alexa Fluor® 488) (ab156033)

Overview

  • Product name
    Anti-Frataxin antibody [18A5DB1] (Alexa Fluor® 488)
    See all Frataxin primary antibodies
  • Description
    Mouse monoclonal [18A5DB1] to Frataxin (Alexa Fluor® 488)
  • Conjugation
    Alexa Fluor® 488. Ex: 495nm, Em: 519nm
  • Tested applications
    Suitable for: Flow Cytmore details
  • Species reactivity
    Reacts with: Human
    Does not react with: Mouse, Rat, Cow
  • Immunogen

    Recombinant full length mature protein, corresponding to amino acids 56-210 of Human Frataxin (UniProt: Q16595).

  • Positive control
    • Flow Cyt: HepG2 cells.
  • General notes

    Product was previously marketed under the MitoSciences sub-brand.

    Alexa Fluor® is a registered trademark of Molecular Probes, Inc, a Thermo Fisher Scientific Company. The Alexa Fluor® dye included in this product is provided under an intellectual property license from Life Technologies Corporation. As this product contains the Alexa Fluor® dye, the purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). As this product contains the Alexa Fluor® dye the sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: in manufacturing; (ii) to provide a service, information, or data in return for payment (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are sold for use in research. For information on purchasing a license to use products containing Alexa Fluor® dyes for purposes other than research, contact Life Technologies Corporation, 5791 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@lifetech.com

Properties

Applications

Our Abpromise guarantee covers the use of ab156033 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
Flow Cyt 1/50.

ab171463-Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.

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

Images

  • Overlay histogram showing HepG2 cells stained with ab156033 (red line). The cells were fixed with 4% formaldehyde (10 min) and then permeabilized with 0.1% PBS-Triton X-100 for 15 min. The cells were then incubated in 1x PBS / 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (ab156033, 1/50 dilution) for 30 min at 22°C.

    Isotype control antibody (black line) was Mouse IgG1 - Alexa Fluor® 488 (ab171463) used at the same concentration and conditions as the primary antibody. Unlabelled sample (blue line) was also used as a control.

    Acquisition of >5,000 events were collected using a 50 mW Blue laser (488nm) and 530/30 bandpass filter.

  • Flow cytometric analysis of Human lymphocytes labeling Frataxin with ab156033 at 0.2 µg/ml.
    Lymphocytes were isolated from Control Patients (red), Freidrich Ataxia Carrier Patients (blue), and Friedrich Ataxia Patients (yellow).
    IgG1 Alexa®488 conjugated Isotype control antibody (black at 0.2 µg/ml).

References

ab156033 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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