Key features and details
- Mouse monoclonal [1H1] to MBNL1
- Suitable for: WB, ICC
- Reacts with: Human
- Isotype: IgG1
Product nameAnti-MBNL1 antibody [1H1]
See all MBNL1 primary antibodies
DescriptionMouse monoclonal [1H1] to MBNL1
Tested applicationsSuitable for: WB, ICCmore details
Species reactivityReacts with: Human
Recombinant full length protein corresponding to Human MBNL1.
Database link: Q9NR56
- WB: HeLa and HEK-293 nuclear fractions. ICC: HeLa cells.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.03% Sodium azide
Constituents: 49.97% PBS, 50% Glycerol (glycerin, glycerine)
Concentration information loading...
PurityProtein G purified
Purification notesPurified from Tissue Culture supernatant.
Our Abpromise guarantee covers the use of ab277620 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000 - 1/2000. Predicted molecular weight: 42 kDa.|
FunctionMediates 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 specificityHighly 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 diseasePlays 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 similaritiesBelongs to the muscleblind family.
Contains 4 C3H1-type zinc fingers.
Cellular localizationNucleus. 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
- EXP antibody
- EXP35 antibody
- EXP40 antibody
HeLa cells stained for MBNL1 (green) using ab277620 at 1/1000 dilution in immunocytochemistry. A chicken polyclonal antibody to HSP60 (red) was used at 1/5000 dilution.
Lanes 2-7 : Anti-MBNL1 antibody [1H1] (ab277620) at 1/1000 dilution
Lane 1 : Molecular Weight ladder
Lane 2 : HEK-293 cytosolic fraction
Lane 3 : HEK-293 nuclear fraction
Lane 4 : NIH-3T3 cytosolic fraction
Lane 5 : NIH-3T3 nuclear fraction
Lane 6 : HeLa cytosolic fraction
Lane 7 : HeLa nuclear fraction
Predicted band size: 42 kDa
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab277620 has not yet been referenced specifically in any publications.