製品の概要

  • 製品名Anti-Frataxin antibody
    Frataxin 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to Frataxin
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Rat
    交差が予測される動物種: 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).

  • ポジティブ・コントロール
    • Rat heart lysate

製品の特性

アプリケーション

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.

アプリケーション Abreviews 特記事項
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.

ターゲット情報

  • 機能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.
  • 組織特異性Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
  • 関連疾患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.
  • 配列類似性Belongs to the frataxin family.
  • 翻訳後修飾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.
  • 細胞内局在Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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 画像

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

    Predicted band size : 23 kDa

Anti-Frataxin antibody (ab113992) 使用論文

ab113992 has not yet been referenced specifically in any publications.

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