製品の概要

  • 製品名
    Anti-Muscleblind-like 1 antibody
    Muscleblind-like 1 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal to Muscleblind-like 1
  • アプリケーション
    適用あり: ELISA, WBmore details
  • 種交差性
    交差種: Mouse, Rat, Human
  • 免疫原

    Recombinant full length human Muscleblind-like 1 protein (AAH50535) with a 26 kDa tag

  • ポジティブ・コントロール
    • HeLa cell lysate; recombinant full length human Muscleblind-like 1 (the immunogen).
  • 特記事項

    Abcam is committed to meeting high standards of ethical manufacturing and has decided to discontinue this product by June 2019 as it has been generated by the ascites method. We are sorry for any inconvenience this may cause. We would recommend antibody ab108519 as a replacement.

製品の特性

  • 製品の状態
    Liquid
  • 保存方法
    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
  • バッファー
    Preservative: None
    Constituents: 1X PBS, pH 7.2
  • Concentration information loading...
  • 精製度
    Protein A purified
  • ポリ/モノ
    モノクローナル
  • アイソタイプ
    IgG1
  • 軽鎖の種類
    kappa
  • 研究分野

アプリケーション

Our Abpromise guarantee covers the use of ab77017 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
ELISA Use at an assay dependent dilution.
WB Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 41 kDa.

ターゲット情報

  • 機能
    Mediates pre-mRNA alternative splicing regulation. Acts either as activator or repressor of splicing on specific pre-mRNA targets. Inhibits cardiac troponin-T (TNNT2) pre-mRNA exon inclusion but induces insulin receptor (IR) pre-mRNA exon inclusion in muscle. Antagonizes the alternative splicing activity pattern of CELF proteins. Regulates the TNNT2 exon 5 skipping through competition with U2AF2. Inhibits the formation of the spliceosome A complex on intron 4 of TNNT2 pre-mRNA. Binds to the stem-loop structure within the polypyrimidine tract of TNNT2 intron 4 during spliceosome assembly. Binds to the 5'-YGCU(U/G)Y-3'consensus sequence. Binds to the IR RNA. Binds to expanded CUG repeat RNA, which folds into a hairpin structure containing GC base pairs and bulged, unpaired U residues.
  • 組織特異性
    Highly expressed in cardiac, skeletal muscle and during myoblast differentiation. Weakly expressed in other tissues (at protein level). Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
  • 関連疾患
    Plays a role in the pathogenesis of dystrophia myotonica type 1 (DM1) [MIM:160900]. A muscular disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness and cardiac arrhythmias. Note=In muscle cells from DM1 patients, MBNL1 is sequestered by DMPK RNAs containing CUG triplet repeat expansions. MBNL1 binding is proportional to repeat length consistent with the direct correlation between the length of repeat expansion and disease severity.
  • 配列類似性
    Belongs to the muscleblind family.
    Contains 4 C3H1-type zinc fingers.
  • 細胞内局在
    Nucleus. Cytoplasm. Cytoplasmic granule. Localized with DDX1, TIAL1 and YBX1 in stress granules upon stress. Localized in the cytoplasm of multinucleated myotubes. Colocalizes with nuclear foci of retained expanded-repeat transcripts in myotubes from patients affected by myotonic dystrophy.
  • Information by UniProt
  • 参照データベース
  • 別名
    • EXP antibody
    • EXP35 antibody
    • EXP40 antibody
    • EXP42 antibody
    • KIAA0428 antibody
    • MBNL antibody
    • MBNL protein antibody
    • MBNL1 antibody
    • MBNL1_HUMAN antibody
    • Muscleblind 41kD isoform antibody
    • Muscleblind like antibody
    • Muscleblind like protein 1 antibody
    • Muscleblind like splicing regulator 1 antibody
    • Muscleblind-like protein 1 antibody
    • Triplet expansion RNA binding protein antibody
    • Triplet-expansion RNA-binding protein antibody
    see all

画像

  • Anti-Muscleblind-like 1 antibody (ab77017) at 5 µg/ml + recombinant tagged full length human Muscleblind-like 1 at 0.2 µg

    Secondary
    Goat anti-Mouse IgG (H&L)-HRP at 1/5000 dilution

    Predicted band size : 41 kDa
    Observed band size : 64 kDa (why is the actual band size different from the predicted?)
  • Anti-Muscleblind-like 1 antibody (ab77017) at 1/500 dilution + HeLa cell lysate at 25 µg

    Secondary
    Goat anti-Mouse IgG (H&L)-HRP at 1/2500 dilution

    Predicted band size : 41 kDa
    Observed band size : 41 kDa

プロトコール

参考文献

ab77017 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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