Anti-Dystrophin 抗体 [MANDRA1] (ab7164)

Reviews (1)Q&A (7)References (18)
Immunohistochemistry (Frozen sections) - Anti-Dystrophin antibody [MANDRA1] (ab7164)

    Key features and details

    • Mouse monoclonal [MANDRA1] to Dystrophin
    • Suitable for: IHC-Fr
    • Reacts with: Human
    • Isotype: IgG1

    製品の概要

    • 製品名

      Anti-Dystrophin antibody [MANDRA1]
      Dystrophin 一次抗体 製品一覧

    • 製品の詳細

      Mouse monoclonal [MANDRA1] to Dystrophin

    • 由来種

      Mouse

    • アプリケーション

      適用あり: IHC-Frmore details
      適用なし: IHC-P

    • 種交差性

      交差種: Human

    • 免疫原

      Recombinant fragment within Human Dystrophin aa 3200-3700. The exact immunogen sequence used to generate this antibody is proprietary information. If additional detail on the immunogen is needed to determine the suitability of the antibody for your needs, please contact our Scientific Support team to discuss your requirements.

    • エピトープ

      128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (a.a. residues 3558-3684).

    • ポジティブ･コントロール

      • lympho blastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells

    • 特記事項

      The C-terminal domain of the human dystrophin molecule (a.a. residues 3558-3684) is present in normal muscle tissue. It is also present in nearly all Becker muscular dystrophies, but is absent in cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx).

      This product was changed from ascites to tissue culture supernatant on 17 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

    製品の特性

    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ab7164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-Fr
    Use at an assay dependent concentration.
    特記事項
    IHC-Fr
    Use at an assay dependent concentration.
  • 追加情報
    Is unsuitable for IHC-P.

    • ターゲット情報

    • 機能

      Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission.

    • 組織特異性

      Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Expressed in brain, muscle, kidney, lung and testis. Isoform 5 is expressed in heart, brain, liver, testis and hepatoma cells. Most tissues contain transcripts of multiple isoforms, however only isoform 5 is detected in heart and liver.

    • 関連疾患

      Defects in DMD are the cause of Duchenne muscular dystrophy (DMD) [MIM:310200]. DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment.
      Defects in DMD are the cause of Becker muscular dystrophy (BMD) [MIM:300376]. BMD resembles DMD in hereditary and clinical features but is later in onset and more benign.
      Defects in DMD are a cause of cardiomyopathy dilated X-linked type 3B (CMD3B) [MIM:302045]; also known as X-linked dilated cardiomyopathy (XLCM). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.

    • 配列類似性

      Contains 2 CH (calponin-homology) domains.
      Contains 22 spectrin repeats.
      Contains 1 WW domain.
      Contains 1 ZZ-type zinc finger.

    • 細胞内局在

      Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
    • Information by UniProt

    • 参照データベース

      • 別名

        • BMD antibody
        • CMD3B antibody
        • DMD antibody
        • DMD_HUMAN antibody
        • Duchenne muscular dystrophy protein antibody
        • Dystrophin antibody
        • Muscular dystrophy Duchenne and Becker types antibody
        see all

      画像

      • Immunohistochemistry (Frozen sections) - Anti-Dystrophin antibody [MANDRA1] (ab7164)
        Immunohistochemistry (Frozen sections) - Anti-Dystrophin antibody [MANDRA1] (ab7164)

        ab7164 staining Dystrophin in frozen human tongue tissue sections by Immunohistochemistry (IHC - Fr- Frozen sections). Samples were incubated 1:100 dilution. A Goat Anti-mouse, FITC- conjugate was used as the secondary antibody. 

        This image was generated using the ascites version of the product.

      参考文献 (18)

      ab7164 を使用した論文を発表された方は、こちらまでお知らせください。データシートに掲載させていただきます。

      ab7164 は 18 報の論文で使用されています。

      • Olie CS  et al. Cytoskeletal disorganization underlies PABPN1-mediated myogenic disability. Sci Rep 10:17621 (2020). PubMed: 33077830
      • Uezu A  et al. Essential role for InSyn1 in dystroglycan complex integrity and cognitive behaviors in mice. Elife 8:N/A (2019). PubMed: 31829939
      • Baruffaldi F  et al. Dynamic Phosphorylation of the Myocyte Enhancer Factor 2Ca1 Splice Variant Promotes Skeletal Muscle Regeneration and Hypertrophy. Stem Cells 35:725-738 (2017). PubMed: 27612437
      • Sekulic-Jablanovic M  et al. Functional characterization of orbicularis oculi and extraocular muscles. J Gen Physiol 147:395-406 (2016). PubMed: 27069119
      • Karolczak J  et al. Myosin VI in skeletal muscle: its localization in the sarcoplasmic reticulum, neuromuscular junction and muscle nuclei. Histochem Cell Biol 139:873-85 (2013). PubMed: 23275125
      View all Publications for this product

      レビューと Q&A

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      1-8 of 8 Abreviews or Q&A

      Western blot abreview for Anti-Dystrophin antibody [MANDRA1]

       Excellent
      Abreviews
      abreview image
      Application
      Western blot
      Sample
      Mouse Cell lysate - whole cell (mouse myotubes)
      Loading amount
      30 µg
      Specification
      mouse myotubes
      Gel Running Conditions
      Reduced Denaturing (4-20% gradient gel)
      Blocking step
      BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
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      Abcam user community

      Verified customer

      投稿 Jul 21 2011

      Question


      In the diagnostic field, it is important to know which antibody is against to the carboxyl, the NH2 or the rod domain. However, It is not clear to me and to some customers (because I reviewed the answers posted in the website) the bunch of antibodies you offer are directed to.

      It is mentioned they share reactivity to the transmembrane part or are associated or share affinity to the other dystrophin associated proteins, but specifically the question is not answered.

      I wonder if you do not mind, to explain me the specific affinity of the antibody (rod, NH2, COOH domains) the antibodies are directed.

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      Answer

      Thank you for your inquiry.


      Based on its primary structure, the dystrophin can be divided into four distinct structural domains: (1) the N-terminal actin-binding domain (aa 14-240), (2) the large triple helical spectrin-like domain (aa 253-3040) composed of 24 repeating units similar to those in β-spectrin, which are predicted to form triple-helical coiled-coils, (3) the cysteine-rich domain (aa 3080- 3360) and the C-terminal domain (aa 3361- 3685). http://www.sciencedirect.com/science/article/pii/0092867488903832


      Please find information on the location of the immunogens by antibody below:


      ab15278: C-terminal

      ab154168: C-terminal

      ab129996: epitope not mapped to our knowledge

      ab14452: epitope not mapped to our knowledge

      ab85302: spectrin-like domain/ rod

      ab7164: C-terminal

      ab15277: C-terminal


      Please note the above antibodies are tested and guaranteed for different species and applications. Please choose carefully. If you let me know what species your samples are from and what application you are planning I will be happy to help choose the most suitable antibody.


      Importantly, I also have to confirm that all our products are for research purposes only and are not intended for diagnostic or therapeutic use.

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      Question

      I would like to know the epitope sequences of these antibodies.

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      Answer

      Thank you for your phone enquiries and interest in our products.

      ab7163 - Anti-Dystrophin antibody [MANDYS8]

      This antibody Monoclonal Anti-Dystrophin, clone MANDYS8, recognizes an epitope located on the rod domain of the human dystrophin molecule, The publications that are cited in the data sheet, which mapped the epitope that the MANDYS8 clone recognises are:

      1. Nguyen, thi Man, et al., FEBS Lett., 262, 237 (1990).
      2. Sedgwick, S., et al. Rapid mapping by transposon mutagenesis of epitopes on the muscular dystrophy protein, dystrophin Nucl. Acids Res. (1991) 19(21): 5889-5894


      According to these references the epitope recognised by this clone lies between residues 1400 and 1505.

      Regarding the epitopes of the other 3 antibodies, ab3149, ab7164, ab15277 - no information is available regarding yet. As soon as we have further details, we will update the corresponding datasheets.

      I hope that this helps and please do not hesitate to contact us if you require any further information or assistance.

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      Question

      I get staining in my cells when treated with the primary antibody but no secondary antibody.

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      Answer

      Thank you for your phone call. As we discussed, this antibody is not conjugated so it should not produce any signal in the absence of a secondary antibody. It is likely that the signal you are observing is autofluorescence. The attached reference details several common types of autofluorescence and tips for avoiding them. I hope this helps, please let me know if you have any additional questions.

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      Question

      Thank you for your very efficient dealingwith my concern regarding the nNOS antibody (see below) for frozen tissue sections. We did get in exchange the otherantibody I had referred to, and it is working both for frozen sections and western blots. The MHC II antibody for western blots, that wepurchased around the same time, also worked very well. So, we will continue orderingthese antibodies.

      However,we have a serious problem now with the dystrophin antibody MANDRA1, ab7164 which you advertise for western blots.In our hands, itdid not work for about 80 ug protein from myofibers in wild type mouse skeletal muscle homogenates, where MHC II is working very fine, even with 0.5 ug protein.

      I would like touse what we paid for the MANDRA1 (ab7164) as a credit for testing an alternative antibody. I have identified ab15277 (rabbit polyclonal) as of potential interest, even if no western blot is shown (although the recommended dilution is 1/100), because it seems to be widely used. The alternative seems to be ab3149 (mouse monoclonal). It does show a nice band, but it does not tell whether it isfrom a skeletal muscle extract, what amount of protein and what antibody dilution.

      Can you please e-mail me to advise on any of these or alternative antibodies for mouse dystrophin in western blot of skeletal muscle homogenates. I would like to talk to you on the phone.

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      Answer

      Thank you for your email. It was nice to speak to you over the phone.
      I am sorry to hear that you are encountering problems with ab7164.

      I am sorry this product did not perform as stated on the datasheet and for the inconvenience this has caused. As requested, I have issued a free of charge replacement with the order number 1017104 with ab3149.

      To check the status of the order please contact our Customer Service team and reference this number.

      Please note that this free of charge replacement vial is also covered by our Abpromise guarantee. Should you still be experiencing difficulties, or if you have any further questions, please do not hesitate to let us know.

      I wish you the best of luck with your research.

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      Question

      Thank you for the information, but the problem is that I want to stain cultured cells instead of sections. Unlike these papers were the authors use cryosections, I have to fix the cells with something. Is this possible? and which fixative do you recommend? Regards,

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      Answer

      Thank you for your e-mail. I would recommend using the same fixative that have been used in the references where staining was done on frozen section; unfortunately I cannot access those papers myself to find this information. I would suspect the fixation will be ice cold acetone or methanol (or a mixture) for 10min, so something similar would be a good starting point. If you have enough cells I would recommend trying in parallel a well with 4%PFA fixing (for 10min) just in case this works in ICC. I hope this will help you, could you please let me know how you get on and do not hesitate to contact me if you need further advice on your staining protocol,

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      Question

      I'm interested in the ab7164 mandra-1 dystrophin antibody. However, I've one question, this is written in the datasheet 'The epitope recognized by the antibody is sensitive to formalin fixation and paraffin embedding'. I want to use this antibody on fixed cells and wonder if pfa fixation is suitable in this case. If not, what do you recommend me?

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      Answer

      Thank you for your enquiry. The antibody will work in frozen sections and I would recommend the following references as sources of staining protocols as they have tested the antibody in IHC: Ellis JM et al. Specificity of dystrophin analysis improved with monoclonal antibodies. Lancet 336:881-2 (1990). PubMed: 1699095. Nguyen TM et al. Monoclonal antibodies for dystrophin analysis. Epitope mapping and improved binding to SDS-treated muscle sections. Biochem J 288 (Pt 2):663-8 (1992). PubMed: 1281410 Gussoni E et al. Normal dystrophin transcripts detected in Duchenne muscular dystrophy patients after myoblast transplantation. Nature 356:435-8 (1992). PubMed: 1557125 I hope these references will help, please let me know if you need further information,

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      Question

      What is recommended as a positive control for this antibody in ELISA?

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      Answer

      Thank you for your phone call. This clone was tested for application in both direct and indirect ELISA as per the citations below (the PubMed links can be found on the online datasheet for ab7164). Morris GE et al. A quantitative ELISA for dystrophin. J Immunol Methods 161:23-8 (1993). PubMed: 8486926 Nguyen TM et al. Monoclonal antibodies for dystrophin analysis. Epitope mapping and improved binding to SDS-treated muscle sections. Biochem J 288 (Pt 2):663-8 (1992). PubMed: 1281410 These references may offer additional information pertaining to appropriate controls and methodology. If you have any additional questions, please contact us again.

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