Anti-MBNL1 抗体 (ab45899)
Key features and details
- Rabbit polyclonal to MBNL1
- Suitable for: IHC-P, ICC/IF, WB
- Reacts with: Human
- Isotype: IgG
製品の概要
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製品名
Anti-MBNL1 antibody
MBNL1 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to MBNL1 -
由来種
Rabbit -
アプリケーション
適用あり: IHC-P, ICC/IF, WBmore details -
種交差性
交差種: Human
交差が予測される動物種: Mouse, Rat
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免疫原
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ポジティブ･コントロール
- Recombinant Human MBNL1 protein (ab114825) can be used as a positive control in WB. This antibody gave a positive signal in the following Human Lysates: Jurkat Whole Cell Jurkat Nuclear Lysate Human sketelal muscle FFPE tissue sections
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特記事項
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
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 +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
バッファー
pH: 7.40
Preservative: 0.02% Sodium azide
Constituents: 98.98% PBS, 1% BSA
Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help. -
Concentration information loading... -
精製度
Immunogen affinity purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
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Compatible Secondaries
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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ab45899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 |
|---|---|---|
| IHC-P | (1) |
Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
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| ICC/IF | (2) |
Use a concentration of 1 µg/ml.
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| WB |
Use a concentration of 1 µg/ml. Detects a band of approximately 35,40,42 kDa (predicted molecular weight: 35,40,42 kDa).
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| 特記事項 |
|---|
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IHC-P
Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. |
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ICC/IF
Use a concentration of 1 µg/ml. |
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WB
Use a concentration of 1 µg/ml. Detects a band of approximately 35,40,42 kDa (predicted molecular weight: 35,40,42 kDa). |
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
- Entrez Gene: 282635 Rat
- Omim: 606516 Human
- SwissProt: Q9NR56 Human
- SwissProt: Q9JKP5 Mouse
- Unigene: 201858 Human
- Unigene: 725347 Human
see all -
別名
- EXP antibody
- EXP35 antibody
- EXP40 antibody
see all
画像
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Immunocytochemistry/ Immunofluorescence - Anti-MBNL1 antibody (ab45899)
ab45899 staining MBNL1 in MCF7 cells. The cells were fixed with 100% methanol (5 min), permeabilized with 0.1% PBS-Triton X-100 for 5 minutes and then blocked with 1% BSA/10% normal goat serum/0.3M glycine in 0.1%PBS-Tween for 1h. The cells were then incubated overnight at 4°C with ab45899 at 1µg/ml and ab7291, Mouse monoclonal [DM1A] to alpha Tubulin - Loading Control. Cells were then incubated with ab150081, Goat polyclonal Secondary Antibody to Rabbit IgG - H&L (Alexa Fluor® 488), pre-adsorbed at 1/1000 dilution (shown in green) and ab150120, Goat polyclonal Secondary Antibody to Mouse IgG - H&L (Alexa Fluor® 594), pre-adsorbed at 1/1000 dilution (shown in pseudocolour magenta). Nuclear DNA was labelled with DAPI (shown in blue).
Image was acquired with a high-content analyser (Operetta CLS, Perkin Elmer) and a maximum intensity projection of confocal sections is shown.
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Western blot - Anti-MBNL1 antibody (ab45899)All lanes : Anti-MBNL1 antibody (ab45899) at 1 µg/ml
Lane 1 : Jurkat (Human T cell lymphoblast-like cell line) Whole Cell Lysate
Lane 2 : Jurkat nuclear extract lysate (ab14844)
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
Performed under reducing conditions.
Predicted band size: 35,40,42 kDa
Observed band size: 35,40,42 kDa why is the actual band size different from the predicted?
Additional bands at: 110 kDa, 52 kDa. We are unsure as to the identity of these extra bands.ab45899 recognises all three known isoforms to MBNL1
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MBNL1 antibody (ab45899)IHC image of ab45899 staining in human skeletal muscle formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab45899, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
プロトコール
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 (19)
ab45899 は 19 報の論文で使用されています。
- Hu N et al. Correction of Clcn1 alternative splicing reverses muscle fiber type transition in mice with myotonic dystrophy. Nat Commun 14:1956 (2023). PubMed: 37029100
- Nelson AR et al. Network model integrated with multi-omic data predicts MBNL1 signals that drive myofibroblast activation. iScience 26:106502 (2023). PubMed: 37091233
- Overby SJ et al. BlockmiR AONs as Site-Specific Therapeutic MBNL Modulation in Myotonic Dystrophy 2D and 3D Muscle Cells and HSALR Mice. Pharmaceutics 15:N/A (2023). PubMed: 37111604
- Dastidar S et al. Comprehensive transcriptome-wide analysis of spliceopathy correction of myotonic dystrophy using CRISPR-Cas9 in iPSCs-derived cardiomyocytes. Mol Ther 30:75-91 (2022). PubMed: 34371182
- Gu X et al. Uninterrupted CAG repeat drives striatum-selective transcriptionopathy and nuclear pathogenesis in human Huntingtin BAC mice. Neuron 110:1173-1192.e7 (2022). PubMed: 35114102