概述

  • 产品名称Anti-Frataxin抗体
    参阅全部 Frataxin 一抗
  • 描述
    兔多克隆抗体to Frataxin
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Rat
    预测可用于: Mouse, Rabbit, Horse, Chicken, Guinea pig, Cow, Cat, Dog, Saccharomyces cerevisiae, Drosophila melanogaster, Zebrafish
  • 免疫原

    Synthetic peptide corresponding to a region within internal amino acids 106-155 (

    EFFEDLADKPYTLKDYDVSFGDGVLTIKLGGDLGTYVINKQTPNKQIWLS

    ) of Rat Frataxin (XP_001078791).

  • 阳性对照
    • Rat heart lysate

性能

应用

Our Abpromise guarantee covers the use of ab113992 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 1 µg/ml. Predicted molecular weight: 23 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

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 图像

  • Anti-Frataxin antibody (ab113992) at 1 µg/ml + Rat heart lysate at 10 µg

    Predicted band size : 23 kDa

Anti-Frataxin antibody (ab113992)参考文献

ab113992 has not yet been referenced specifically in any publications.

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