Anti-MBNL1 抗体 [EPR4565] (ab108519)
![Western blot - Anti-MBNL1 antibody [EPR4565] (ab108519)](https://www.abcam.co.jp/ps/products/108/ab108519/Images/ab108519-121235-anti-muscleblind-like-1-antibody-epr4565-western-blot.jpg "Western blot - Anti-MBNL1 antibody [EPR4565] (ab108519)")
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR4565] to MBNL1
- Suitable for: WB
- Reacts with: Human
Related conjugates and formulations
製品の概要
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製品名
Anti-MBNL1 antibody [EPR4565]
MBNL1 一次抗体 製品一覧 -
製品の詳細
Rabbit monoclonal [EPR4565] to MBNL1 -
由来種
Rabbit -
アプリケーション
適用あり: WBmore details
適用なし: Flow Cyt,ICC,IHC-P or IP -
種交差性
交差種: Human
交差が予測される動物種: Mouse
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免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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ポジティブ･コントロール
- Jurkat, HeLa, and A673 cell lysates
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特記事項
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Rat: We have preliminary internal testing data to indicate this antibody may not react with this species. Please contact us for more information.
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
バッファー
pH: 7.20
Preservative: 0.05% Sodium azide
Constituents: 0.1% BSA, 40% Glycerol (glycerin, glycerine), 9.85% Tris glycine, 50% Tissue culture supernatant -
Concentration information loading... -
精製度
Tissue culture supernatant -
ポリ/モノ
モノクローナル -
クローン名
EPR4565 -
アイソタイプ
IgG -
研究分野
関連製品
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Alternative Versions
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Isotype control
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Positive Controls
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Recombinant Protein
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab108519の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
| アプリケーション | Abreviews | 特記事項 |
|---|---|---|
| WB |
1/1000 - 1/10000. Predicted molecular weight: 42 kDa.
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| 特記事項 |
|---|
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WB
1/1000 - 1/10000. Predicted molecular weight: 42 kDa. |
追加情報
Is unsuitable for Flow Cyt,ICC,IHC-P or IP.
ターゲット情報
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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
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参照データベース
- Entrez Gene: 4154 Human
- Entrez Gene: 56758 Mouse
- Omim: 606516 Human
- SwissProt: Q9NR56 Human
- SwissProt: Q9JKP5 Mouse
- Unigene: 201858 Human
- Unigene: 725347 Human
- Unigene: 255723 Mouse
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別名
- EXP antibody
- EXP35 antibody
- EXP40 antibody
see all
画像
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Western blot - Anti-MBNL1 antibody [EPR4565] (ab108519)All lanes : Anti-MBNL1 antibody [EPR4565] (ab108519) at 1/1000 dilution
Lane 1 : Jurkat cell lysate
Lane 2 : HeLa cell lysate
Lane 3 : A673 cell lysate
Lysates/proteins at 10 µg per lane.
Predicted band size: 42 kDa -
![Anti-MBNL1 antibody [EPR4565] (ab108519)](https://www.abcam.co.jp/ps/products/108/ab108519/Images/ab108519-2-benefits-of-recombinant-antibodies.png)
Anti-MBNL1 antibody [EPR4565] (ab108519)
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (7)
ab108519 は 7 報の論文で使用されています。
- Yin Q et al. Dosage effect of multiple genes accounts for multisystem disorder of myotonic dystrophy type 1. Cell Res 30:133-145 (2020). PubMed: 31853004
- Idris M et al. The MBNL/CELF Splicing Factors Regulate Cytosolic Sulfotransferase 4A1 Protein Expression during Cell Differentiation. Drug Metab Dispos 47:314-319 (2019). PubMed: 30606728
- Liu H et al. Alternative splicing analysis in human monocytes and macrophages reveals MBNL1 as major regulator. Nucleic Acids Res 46:6069-6086 (2018). PubMed: 29771377
- Teplyuk NM et al. Therapeutic potential of targeting microRNA-10b in established intracranial glioblastoma: first steps toward the clinic. EMBO Mol Med 8:268-87 (2016). WB ; Human . PubMed: 26881967
- Alves S et al. Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins. Mol Neurodegener 11:58 (2016). IHC . PubMed: 27465358
- Zhang BW et al. miR-30-5p Regulates Muscle Differentiation and Alternative Splicing of Muscle-Related Genes by Targeting MBNL. Int J Mol Sci 17:N/A (2016). WB . PubMed: 26840300
- Screen M et al. Abnormal splicing of NEDD4 in myotonic dystrophy type 2: possible link to statin adverse reactions. Am J Pathol 184:2322-32 (2014). PubMed: 24907641