Anti-MBNL1抗体[1D11] (ab77017)
![Western blot - Anti-MBNL1 antibody [1D11] (ab77017)](https://www.abcam.cn/ps/products/77/ab77017/Images/ab77017-92641-anti-muscleblind-like-1-antibody-western-blot.jpg "Western blot - Anti-MBNL1 antibody [1D11] (ab77017)")
Key features and details
- Mouse monoclonal [1D11] to MBNL1
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG1
概述
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产品名称
Anti-MBNL1抗体[1D11]
参阅全部 MBNL1 一抗 -
描述
小鼠单克隆抗体[1D11] to MBNL1 -
宿主
Mouse -
经测试应用
适用于: WBmore details -
种属反应性
与反应: Human -
免疫原
Recombinant full length protein corresponding to Human MBNL1. (AAH50535)
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阳性对照
- HeLa whole cell lysate (ab150035); recombinant full length human MBNL1 (the immunogen).
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常规说明
This product was changed from ascites to tissue culture supernatant on 30th May 2019. Please note that the dilutions may need to be adjusted accordingly. If you have any questions, please do not hesitate to contact our scientific support team.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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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
pH: 7.40
Constituent: PBS -
Concentration information loading... -
纯度
Tissue culture supernatant -
纯化说明
Purified from TCS. -
克隆
单克隆 -
克隆编号
1D11 -
同种型
IgG1 -
轻链类型
kappa -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab77017于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度；实际最佳的稀释度/浓度应由使用者检定。
| 应用 | Ab评论 | 说明 |
|---|---|---|
| WB |
Use at an assay dependent concentration. Predicted molecular weight: 41 kDa.
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| 说明 |
|---|
|
WB
Use at an assay dependent concentration. Predicted molecular weight: 41 kDa. |
靶标
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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
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数据库链接
- Entrez Gene: 4154 Human
- Omim: 606516 Human
- SwissProt: Q9NR56 Human
- Unigene: 201858 Human
- Unigene: 725347 Human
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别名
- EXP antibody
- EXP35 antibody
- EXP40 antibody
see all
图片
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Western blot - Anti-MBNL1 antibody [1D11] (ab77017)Anti-MBNL1 antibody [1D11] (ab77017) at 5 µg/ml + recombinant tagged full length human MBNL1 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?Molecular weight of the tag alone is 26 kDa.
This image was generated using the ascites version of the product.
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![Western blot - Anti-MBNL1 antibody [1D11] (ab77017)](https://www.abcam.cn/ps/products/77/ab77017/Images/ab77017-92640-anti-muscleblind-like-1-antibody-western-blot.jpg)
Western blot - Anti-MBNL1 antibody [1D11] (ab77017)Anti-MBNL1 antibody [1D11] (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 kDaThis image was generated using the ascites version of the product.
数据表及文件
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Datasheet download
文献 (4)
ab77017 被引用在 4 文献中.
- González-Martínez I et al. Peptide-conjugated antimiRs improve myotonic dystrophy type 1 phenotypes by promoting endogenous MBNL1 expression. Mol Ther Nucleic Acids 34:102024 (2023). PubMed: 37744174
- Moreno N et al. Rapid Determination of MBNL1 Protein Levels by Quantitative Dot Blot for the Evaluation of Antisense Oligonucleotides in Myotonic Dystrophy Myoblasts. Methods Mol Biol 2434:207-215 (2022). PubMed: 35213019
- Rapisarda A et al. Defined D-hexapeptides bind CUG repeats and rescue phenotypes of myotonic dystrophy myotubes in a Drosophila model of the disease. Sci Rep 11:19417 (2021). PubMed: 34593893
- Cerro-Herreros E et al. miR-23b and miR-218 silencing increase Muscleblind-like expression and alleviate myotonic dystrophy phenotypes in mammalian models. Nat Commun 9:2482 (2018). PubMed: 29946070
