Overview

  • Product name
    Anti-Muscleblind-like 1 antibody
    See all Muscleblind-like 1 primary antibodies
  • Description
    Rabbit polyclonal to Muscleblind-like 1
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat, Chicken, Cow
  • Immunogen

    Recombinant fragment, corresponding to a region within amino acids 81-326 of Human Muscleblind-like 1 (Q9NR56).

  • Positive control
    • U-87 MG whole cell lysate.
  • General notes
    Store as concentrated solution.

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • Storage buffer
    pH: 7.00
    Preservative: 0.01% Thimerosal (merthiolate)
    Constituents: 78% PBS, 20% Glycerol, 1% BSA
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab126138 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/500 - 1/3000. Predicted molecular weight: 42 kDa.

Target

  • Function
    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.
  • Tissue specificity
    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.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the muscleblind family.
    Contains 4 C3H1-type zinc fingers.
  • Cellular localization
    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
  • Database links
  • Alternative names
    • 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

Images

  • Anti-Muscleblind-like 1 antibody (ab126138) at 1/1000 dilution + U-87 MG whole cell lysate at 30 µg

    Predicted band size : 42 kDa

References

ab126138 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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