Anti-Frataxin抗体[EPR6107] (ab124680)


  • 产品名称Anti-Frataxin抗体[EPR6107]
    参阅全部 Frataxin 一抗
  • 描述
    兔单克隆抗体[EPR6107] to Frataxin
  • 经测试应用适用于: WBmore details
    不适用于: IHC-P
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) corresponding to Human Frataxin aa 150 to the C-terminus.

  • 阳性对照
    • Human heart, Human fetal skin and PBL lysates.
  • 常规说明

    This product is a recombinant rabbit monoclonal antibody.

    Produced using Abcam’s RabMAb® technology. RabMAb® technology is covered by the following U.S. Patents, No. 5,675,063 and/or 7,429,487.

    We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated ‘PUR’ on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.

    Mouse and Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.



Our Abpromise guarantee covers the use of ab124680 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 1/1000 - 1/10000. Detects a band of approximately 17, 30 kDa (predicted molecular weight: 19, 23 kDa).
  • 应用说明Is unsuitable for IHC-P.
  • 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 [EPR6107] 图像

    • All lanes : Anti-Frataxin antibody [EPR6107] (ab124680) at 1/5000 dilution (purified)

      Lane 1 : Human fetal heart lysate
      Lane 2 : Human skin lysate

      Lysates/proteins at 20 µg per lane.

      Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/2000 dilution

      Predicted band size : 19, 23 kDa
      Observed band size : 30 kDa (why is the actual band size different from the predicted?)
      Blocking and diluting buffer 5% NFDM/TBST
    • All lanes : Anti-Frataxin antibody [EPR6107] (ab124680) at 1/1000 dilution (unpurified)

      Lane 1 : Human heart lysate
      Lane 2 : Human fetal skin lysate
      Lane 3 : PBL lysate

      Lysates/proteins at 10 µg per lane.

      Goat anti-Rabbit HRP at 1/2000 dilution

      Predicted band size : 19, 23 kDa
      Observed band size : 17,30 kDa (why is the actual band size different from the predicted?)
    • Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD

    Anti-Frataxin antibody [EPR6107] (ab124680)参考文献

    ab124680 has not yet been referenced specifically in any publications.

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