Anti-Frataxin抗体[17A11] (ab113691)

概述

性能

应用

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.

应用 Ab评论 说明
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. 1



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.

靶标

  • 功能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.
  • 组织特异性Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
  • 疾病相关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.
  • 序列相似性Belongs to the frataxin family.
  • 翻译后修饰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.
  • 细胞定位Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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

Anti-Frataxin antibody [17A11] 图像

  • 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.
  • 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.

Anti-Frataxin antibody [17A11] (ab113691)参考文献

This product has been referenced in:
  • 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 . Read more (PubMed: 23838345) »

See 1 Publication for this product

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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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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 :
http://www.abcam.com/Frataxi...

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Abcam guarantees this product to work in the species/application used in this Abreview.
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
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Abcam user community

Verified customer

提交于 Apr 16 2012

Thank you for you inquiry.

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

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

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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Thank you for providing that information.

I have issued a free of charge replacement (the new order number is xxxxxx, PO number FOCR xxxxxx). Unfortunately the antibody is currently not in stock and will therefore take 1-2 weeks to be deliver...

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