Anti-Muscleblind-like 1抗体(ab77017)

概述

  • 产品名称Anti-Muscleblind-like 1抗体
    参阅全部 Muscleblind-like 1 一抗
  • 描述
    小鼠单克隆抗体to Muscleblind-like 1
  • 经测试应用适用于: ELISA, WBmore details
  • 种属反应性
    与反应: Mouse, Rat, Human
  • 免疫原

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

  • 阳性对照
    • HeLa cell lysate; recombinant full length human Muscleblind-like 1 (the immunogen).

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Preservative: None
    Constituents: 1X PBS, pH 7.2
  • Concentration information loading...
  • 纯度Protein A purified
  • 克隆单克隆
  • 同种型IgG1
  • 轻链类型kappa
  • 研究领域

相关产品

应用

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.

应用 Ab评论 说明
ELISA Use at an assay dependent dilution.
WB Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 41 kDa.

靶标

  • 功能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.
  • 组织特异性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.
  • 疾病相关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.
  • 序列相似性Belongs to the muscleblind family.
    Contains 4 C3H1-type zinc fingers.
  • 细胞定位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
  • 数据库链接
  • 别名
    • 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

Anti-Muscleblind-like 1 antibody 图像

  • 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

Anti-Muscleblind-like 1 antibody (ab77017)参考文献

ab77017 has not yet been referenced specifically in any publications.

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