Anti-Frataxin antibody [17A11] (ab113691)

Reviews (3)Q&A (4)References (7)
Western blot - Anti-Frataxin antibody [17A11] (ab113691)
  • Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [17A11] (ab113691)
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [17A11] (ab113691)
  • Flow Cytometry - Anti-Frataxin antibody [17A11] (ab113691)

Key features and details

  • Mouse monoclonal [17A11] to Frataxin
  • Suitable for: WB, ICC/IF, Flow Cyt, IHC-P
  • Reacts with: Mouse, Rat, Cow, Dog, Human, Recombinant fragment
  • Isotype: IgG1

Overview

  • Product name

    Anti-Frataxin antibody [17A11]
    See all Frataxin primary antibodies

  • Description

    Mouse monoclonal [17A11] to Frataxin

  • Host species

    Mouse

  • Tested applications

    Suitable for: WB, ICC/IF, Flow Cyt, IHC-Pmore details

  • Species reactivity

    Reacts with: Mouse, Rat, Cow, Dog, Human, Recombinant fragment

  • Immunogen

    Recombinant fragment within Human Frataxin aa 1-120. The exact sequence is proprietary.
    Database link: Q16595

  • 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.

    Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.

    Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.

    We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.

    In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.

    We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.

    Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.

    Please check that this product meets your needs before purchasing. If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, as well as customer reviews and Q&As.

Properties

Applications

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.
IHC-P Use a concentration of 10 µg/ml.

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

    see all

  • 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

  • Western blot - Anti-Frataxin antibody [17A11] (ab113691)
    Western blot - Anti-Frataxin antibody [17A11] (ab113691)
    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

  • Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [17A11] (ab113691)
    Immunocytochemistry/ Immunofluorescence - Anti-Frataxin antibody [17A11] (ab113691)
    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.
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [17A11] (ab113691)
    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [17A11] (ab113691)

    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.

  • Flow Cytometry - Anti-Frataxin antibody [17A11] (ab113691)
    Flow Cytometry - Anti-Frataxin antibody [17A11] (ab113691)
    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.

References (7)

Publishing research using ab113691? Please let us know so that we can cite the reference in this datasheet.

ab113691 has been referenced in 7 publications.

  • Weng L  et al. Evaluation of antibodies for western blot analysis of frataxin protein isoforms. J Immunol Methods 474:112629 (2019). PubMed: 31279523
  • 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). PubMed: 29447225
  • Kemp KC  et al. Bone marrow transplantation stimulates neural repair in Friedreich's ataxia mice. Ann Neurol 83:779-793 (2018). PubMed: 29534309
  • Abeti R  et al. Calcium Deregulation: Novel Insights to Understand Friedreich's Ataxia Pathophysiology. Front Cell Neurosci 12:264 (2018). PubMed: 30333728
  • Guo L  et al. Characterization of a new N-terminally acetylated extra-mitochondrial isoform of frataxin in human erythrocytes. Sci Rep 8:17043 (2018). PubMed: 30451920
  • 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 . PubMed: 27148589
  • Oglesbee D  et al. High-throughput immunoassay for the biochemical diagnosis of Friedreich ataxia in dried blood spots and whole blood. Clin Chem 59:1461-9 (2013). ELISA ; Human . PubMed: 23838345

Customer reviews and Q&As

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1-7 of 7 Abreviews or Q&A

Western blot abreview for Anti-Frataxin antibody [17A11]

 Good
Abreviews
abreview image
Application
Western blot
Sample
Human Cell lysate - whole cell (HEPG2)
Gel Running Conditions
Reduced Denaturing (4-12% Bis-Tris)
Loading amount
40 µg
Specification
HEPG2
Blocking step
Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Read More

Abcam user community

Verified customer

Submitted Oct 19 2018

Immunocytochemistry/ Immunofluorescence abreview for Anti-Frataxin antibody [17A11]

 Excellent
Abreviews
abreview image
Application
Immunocytochemistry/ Immunofluorescence
Sample
Human Cell (HEK293)
Permeabilization
Yes - 0.1% Triton X-100, 10 min RT
Specification
HEK293
Blocking step
Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Fixative
Paraformaldehyde
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Abcam user community

Verified customer

Submitted Jun 05 2018

Question

Our customer is looking for anti human fartaxin for WB and ICC/IF. She needs this antibody to recognize the sequence that starts at amino acid no. 81 (and everything up to the end of C'). Kindly advise if ab110328 and/or ab113691 will be suitable for this purpose. Thanks in advance for your assistance and reply. Kind Regards,

Read More
Answer

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 unable to determine whether they will recognise the sequence mentioned.
We do have other alternative products that you may want to consider:
https://www.abcam.com/index.html?pageconfig=searchresults&search=Frataxin&pt=1&sd=2&fViewMore=1
If you need further information about any of them, please do not hesitate to contact me back.

Read More

Question

I have bought with their anti-frataxin monoclonal antibody (17A11) (from 113 691) and served in various rat models. Now I have bands at 38kDa (actually they should be at 17 kDa), you could tell me first what could be the problem?

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Answer

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 hören, dass Sie Probleme mit diesem Antikörper haben. Wir haben zur Zeit keinen Grund zur Annahme, dass es ein Problem haben sollte mit diesem Antikörper. Ich kann Ihnen daher ohne weiteren Information nicht sagen, woher das Problem kommt. Ich kann Ihnen jedoch versichern, dass der Antikörper auf Ratte und im Western blot getestet wurde, und auch von uns so garantiert wird.

Es könnte zum Beispiel sein, dass die Bande bei 38kD ein Komplex ist. In diesem Fall sollte man die Probendenaturieren und reduzieren. Ich würde mir jedoch das Resultat gerneetwas genauer anschauen und bräuchte dazujedochmehr Informationenüber Ihre Proben und Ihre Protokoll zu senden.Ich habe daher unseren Fragebogen als Word-Dokument an diese E-Mail angehängt. Durch das Ausfüllen des Fragebogens erhalten wir alle nötigen Informationen über Ihre Proben und Ihr Protokoll. Sobald Sie dieses Formular an uns zurückgeschickt haben, kann ich mir Ihr Protokoll genauer ansehen und möglichst Veränderungsvorschläge machen, die Ihre Ergebnisse verbessern werden. Falls sich herausstellt, dass der Antikörper nicht so funktioniert, wie auf dem Datenblatt beschrieben und er innerhalb der letzten 180 Tage gekauft wurde, werden wir Ihnen gerne einen Ersatz oder eine Gutschrift schicken.

Ich freue mich, bald wieder von Ihnen zu hören.

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Western blot abreview for Anti-Frataxin antibody [17A11]

 Good
Abreviews
abreview image
Application
Western blot
Sample
Rat Tissue lysate - whole (Liver)
Loading amount
50 µg
Specification
Liver
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
Read More

Abcam user community

Verified customer

Submitted Apr 16 2012

Question

What is the recommended in cell ELISA protocol?

Read More
Answer

Thank you for you inquiry.

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

https://www.abcam.com/index.html?pageconfig=resource&rid=14736

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

Read More

Question

Dear Sir/Madam

Thank you very much for your email and pursuing our antibody issue. I would like to try an alternativeabcam antibody tofrataxin -ab113992 -as until this recent problem we have always found theadcam antibodies to work well.

The order numberfor our originalfrataxinantibody wasAV1029152 - ordered on the 3/1/12 to be delivered to


Many thanks for all your help

Read More
Answer

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 tissue lysate but not in the mitochondrial fraction.

Is the secondary antibody pre-adsorbed to reduce reactivity with rat IgG?

I think it might be worth trying a gel where you only load the mitochondrial fraction as this would allow a longer exposure time without the IgG bands (if that is what they are). If running the whole tissue lysate it may also be worth removing the reducing agent from the sample to make the antibody remain at the top of the blot.

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