• Product name
    Anti-Frataxin antibody [17A11]
    See all Frataxin primary antibodies
  • Description
    Mouse monoclonal [17A11] to Frataxin
  • Host species
  • Tested applications
    Suitable for: WB, ICC/IF, Flow Cyt, In-Cell ELISA, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Cow, Dog, Human
  • Immunogen

    Recombinant full length protein

  • Positive control
    • WB: heart, liver tissue lysate. IHC-P: heart muscle, liver, kidney tissue (FFPE).
  • General notes

    This antibody clone is manufactured by Abcam.

    Product was previously marketed under the MitoSciences sub-brand.

    If you require this antibody in a particular buffer formulation or a particular conjugate for your experiments, please contact orders@abcam.com or you can find further information here.



Our Abpromise guarantee covers the use of ab113691 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 4 µg/ml. Detects a band of approximately 14 kDa (predicted molecular weight: 14 kDa).
ICC/IF Use a concentration of 1 µg/ml.
Flow Cyt Use a concentration of 1 µg/ml.

ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.

In-Cell ELISA Use a concentration of 1 µg/ml.
IHC-P Use a concentration of 10 µg/ml.


  • 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
    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


  • All lanes : Anti-Frataxin antibody [17A11] (ab113691)

    Lane 1 : Recombinant frataxin
    at 0.1 µg
    Lane 2 : Human heart mitochondria
    at 15 µg
    Lane 3 : Human liver mitochondria
    at 15 µg
    Lane 4 : HepG2 whole cell lysate
    at 15 µg
    Lane 5 : Bovine heart mitochondria
    at 15 µg
    Lane 6 : H9C2 rat whole cell lysate
    at 15 µg
    Lane 7 : MEF mouse embryonic fibroblast
    at 15 µg
    Lane 8 : Mouse liver mitochondria
    at 15 µg
    Lane 9 : Rat liver mitochondria

    at 15 µg

    Predicted band size: 14 kDa

  • ab113691 stained fibroblast cells. The cells were paraformaldehyde fixed (4%, 20 min) and Triton X-100 permeabilized (0.1%, 15min). The cells were incubated with the antibody (ab113691, 1µg/ml) for 2h at room temperature or over night at 4°C. The secondary antibody was (green) Alexa Fluor® 488 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1h. 10% Goat serum was used as the blocking agent for all blocking steps. The target protein locates to the mitochondrial matrix.
  • IHC image of Frataxin staining in Human normal heart muscle formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab113691, 10µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

    For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.

  • Normal human lymphoblast cells were stained with 1 µg/mL ab113691 (blue) or an equal amount of an isotype control antibody (red) and analyzed by flow cytometry.


This product has been referenced in:
  • Wang Q  et al. Low apolipoprotein A-I levels in Friedreich's ataxia and in frataxin-deficient cells: Implications for therapy. PLoS One 13:e0192779 (2018). Read more (PubMed: 29447225) »
  • Joshi M  et al. Mutations in the substrate binding glycine-rich loop of the mitochondrial processing peptidase-a protein (PMPCA) cause a severe mitochondrial disease. Cold Spring Harb Mol Case Stud 2:a000786 (2016). Human . Read more (PubMed: 27148589) »

See all 3 Publications for this product

Customer reviews and Q&As

Immunocytochemistry/ Immunofluorescence
Human Cell (HEK293)
Yes - 0.1% Triton X-100, 10 min RT
Blocking step
Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C

Abcam user community

Verified customer

Submitted Jun 05 2018

A refund has been issued.

Hello! Only one of these antibodies are available as a separate component from the kit, ab113691. Please let me know if you have any further questions.

Thank you for contacting us. I apologize for the waiting.
Both antibodies were raised against the mature protein: Recombinant full length Human Frataxin (amino acids 56-210). Unfortunately we have not done epitope mapping and therefore we are unab...

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Vielen Dank dafür, dass Sie sich die Zeit genommen haben, unseren Fragebogen auszufüllen.

Es tut mir leid, dass Sie Probleme mit unserem Antikörper hatten.

Um Ihr Problem zu lösen, möchte ich die folgenden...

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Wir haben in der Tat auch einen deutschen Kundenservice, meine Kollegin hat nur nicht erkannt, dass Sie in Deutsch geschrieben hatten! Entschuldigen Sie bitte die Umstände.

Vielen Dank fürIhre Anfrage.

Es tut mir leid zu ...

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Thank you for contacting us.
The email sent to you on March 14 was:
"I have confirmed the following with the laboratory:
For blotting experiments compatible with these kits you are going to want to use :

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Abcam guarantees this product to work in the species/application used in this Abreview.
Western blot
Rat Tissue lysate - whole (Liver)
Loading amount
50 µg
Gel Running Conditions
Reduced Denaturing (12%)
Blocking step
Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 20°C

Abcam user community

Verified customer

Submitted Apr 16 2012

Thank you for you inquiry.

Below you can find the link to our recommended in cell ELISA protocol:


I hope this information helps. Please contact us with any...

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I have been having another think about the results you have been seeing. Could it be that the bands you are observing arerat IgG? This would correlate to the band sizes observed (˜30 kDa and 50 kDa?) and the fact that you are seeing it in the whole tis...

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1-10 of 13 Abreviews or Q&A


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