Anti-Frataxin抗体[18A5DB1] (ab110328)


  • 产品名称Anti-Frataxin抗体[18A5DB1]
    参阅全部 Frataxin 一抗
  • 描述
    小鼠单克隆抗体[18A5DB1] to Frataxin
  • 经测试应用适用于: WB, ICC/IF, Flow Cyt, IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant full length mature protein, corresponding to amino acids 56-210 of Human Frataxin.

  • 阳性对照
    • Recombinant Human Frataxin; normal Human fibroblasts (MRC5); HL60 cells. This antibody gave a positive result in IHC in the following FFPE tissue: Human colon adenocarcinoma.
  • 常规说明

    Product was previously marketed under the MitoSciences sub-brand.

    “Although this antibody does work in WB, we have found that ab113691 typically gives a more robust signal. Therefore, if you plan on using this antibody in WB, we recommend ab113691 as an alternative option.”



Our Abpromise guarantee covers the use of ab110328 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 3 - 5 µg/ml. Predicted molecular weight: 23 kDa. (A chemiluminescent detection system is highly recommended)
ICC/IF Use a concentration of 0.5 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.


  • 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 [18A5DB1] 图像

  • IHC image of Frataxin staining in Human colon adenocarcinoma 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 ab110328, 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.

  • Immunocytochemistry analysis using ab110328 at 0.5µg/ml staining Frataxin in normal Human fibroblasts (MRC5) (fixed and permeabilized) followed by an AlexaFluor® 594-conjugated-goat-anti-mouse IgG1 isotype specific secondary antibody (2 µg/ml). .
  • Flow cytometric analysis using ab110328 at 1µg/ml staining Frataxin in HL60 cells (blue). Isotype control antibody (red).
  • Anti-Frataxin antibody [18A5DB1] (ab110328) at 5 µg/ml + Recombinant Human Frataxin at 0.0005 µg

    Predicted band size : 23 kDa

Anti-Frataxin antibody [18A5DB1] (ab110328)参考文献

This product has been referenced in:
  • Distante S  et al. Blood removal therapy in hereditary hemochromatosis induces a stress response resulting in improved genome integrity. Transfusion 56:1435-41 (2016). Read more (PubMed: 27045387) »
  • Palandri A  et al. Frataxin inactivation leads to steroid deficiency in flies and human ovarian cells. Hum Mol Genet 24:2615-26 (2015). WB . Read more (PubMed: 25628335) »

See all 16 Publications for this product

Product Wall

Application Western blot
Loading amount 30 µg
Gel Running Conditions Reduced Denaturing (13 % separating gel 5 % collecting gel)
Sample Human Cell lysate - other (see additional notes)
Specification see additional notes
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: RT°C

Abcam user community

Verified customer

提交于 May 22 2014

Application Immunocytochemistry/ Immunofluorescence
Blocking step BSA as blocking agent for 15 minute(s) · Concentration: 1% · Temperature: 20°C
Sample Human Cell (SH-SY5Y human neuroblastoma)
Specification SH-SY5Y human neuroblastoma
Permeabilization Yes - 0.2% NP-40, 3min
Fixative Formaldehyde

Dr. George Allen

Verified customer

提交于 Dec 16 2013

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Human Cell lysate - whole cell (fibroblasts)
Loading amount 20 µg
Specification fibroblasts
Gel Running Conditions Reduced Denaturing (4-12%)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C

Abcam user community

Verified customer

提交于 Apr 12 2013

Thank you for your email, I am sorry for the delay in getting back to you.

I have a couple of antibodies your customer may be interested in:

Anti-Frataxin antibody [18A5DB1] (ab110328)


Anti-ATP5J antibody [10C6AC...

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I am sorry to hear that you have been experiencing problems using this product in the application that you wish.

In order to assess the quality of our products I would ask that you complete a brief questionnaire relating to the application us...

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I am sorry this product did not perform as stated on the datasheet and for the inconvenience this has caused. As requested, I have issued a free of charge replacement for one vial of ab48281.
To check the status of the order please contact our Cus...

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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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Following my previous email, I would like to inform you that all of our products are covered by our Abpromise, which guarantees that the product will work in the applications and species specified on the datasheet, or we will offer a replacement or a c...

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Thank you for contacting us.
Please find attached to this email the recommended flow cytometry protocol. Also below I copied a brief protocol for staining with the anti-Frataxin antibody [18A5DB1] (ref. ab110328). As isotype control I would recomm...

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Thank you for contacting Abcam.

Please find attached the protocol that was used to test ab110328 in flow cytometry.

If there is anything else I can help you with, please let me know.