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
Product nameAnti-Frataxin antibody [17A11]
See all Frataxin primary antibodies
DescriptionMouse monoclonal [17A11] to Frataxin
Tested applicationsSuitable for: WB, ICC/IF, Flow Cyt, IHC-Pmore details
Species reactivityReacts with: Mouse, Rat, Cow, Dog, Human, Recombinant fragment
Recombinant fragment within Human Frataxin aa 1-120. The exact sequence is proprietary.
Database link: Q16595
- WB: heart, liver tissue lysate. IHC-P: heart muscle, liver, kidney tissue (FFPE).
This antibody clone is manufactured by Abcam.
Product was previously marketed under the MitoSciences sub-brand.
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.
Storage instructionsShipped at 4°C. Store at +4°C.
Storage bufferPreservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
Purification notesThe antibody was produced in vitro using hybridomas grown in serum-free medium, and then purified by affinity purification.
Light chain typekappa
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.
|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.|
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.
modificationsProcessed 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
- CyaY antibody
- d-FXN antibody
- FA antibody
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.
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