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リコンビナントRabMAb

Anti-Dystrophin 抗体 [EPR9598(ABC)] (ab154168)

Submit a review Q&A (1)References (19)
Western blot - Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)
  • Western blot - Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)
  • Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)

Key features and details

  • Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
  • Rabbit monoclonal [EPR9598(ABC)] to Dystrophin
  • Suitable for: WB
  • Reacts with: Human

Conjugates logo Related conjugates and formulations

Carrier Free

製品の概要

  • 製品名

    Anti-Dystrophin antibody [EPR9598(ABC)]
    Dystrophin 一次抗体 製品一覧

  • 製品の詳細

    Rabbit monoclonal [EPR9598(ABC)] to Dystrophin

  • 由来種

    Rabbit

  • アプリケーション

    適用あり: WBmore details
    適用なし: ICC/IF or IHC-P

  • 種交差性

    交差種: Human

  • 免疫原

    Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.

  • ポジティブ･コントロール

    • Human fetal muscle; fetal heart lysates; Human fetal skeletal muscle lysates.

  • 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
    For more information see here.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

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ab154168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: 427 kDa.
特記事項
WB
1/1000 - 1/10000. Predicted molecular weight: 427 kDa.
追加情報
Is unsuitable for ICC/IF or 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

    • Western blot - Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)
      Western blot - Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)
      Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168) at 1/1000 dilution (purified) + Human fetal skeletal muscle lysates at 20 µg

      Secondary
      Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution

      Predicted band size: 427 kDa



      Blocking and diluting buffer : 5% NFDM/TBST
    • Western blot - Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)
      Western blot - Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)
      All lanes : Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168) at 1/1000 dilution

      Lane 1 : Human fetal muscle lysate
      Lane 2 : Human fetal heart lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : Goat anti-rabbit HRP at 1/2000 dilution

      Predicted band size: 427 kDa

    • Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)
      Anti-Dystrophin antibody [EPR9598(ABC)] (ab154168)

    参考文献 (19)

    ab154168 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。

    ab154168 は 19 報の論文で使用されています。

    • van Deutekom J  et al. Next Generation Exon 51 Skipping Antisense Oligonucleotides for Duchenne Muscular Dystrophy. Nucleic Acid Ther 33:193-208 (2023). PubMed: 37036788
    • Hiyoshi T  et al. Electrical impedance myography detects dystrophin-related muscle changes in mdx mice. Skelet Muscle 13:19 (2023). PubMed: 37980539
    • Gushchina LV  et al. Persistence of exon 2 skipping and dystrophin expression at 18 months after U7snRNA-mediated therapy in the Dup2 mouse model. Mol Ther Methods Clin Dev 31:101144 (2023). PubMed: 38027058
    • Chesshyre M  et al. Investigating the role of dystrophin isoform deficiency in motor function in Duchenne muscular dystrophy. J Cachexia Sarcopenia Muscle 13:1360-1372 (2022). PubMed: 35083887
    • Wein N  et al. Systemic delivery of an AAV9 exon-skipping vector significantly improves or prevents features of Duchenne muscular dystrophy in the Dup2 mouse. Mol Ther Methods Clin Dev 26:279-293 (2022). PubMed: 35949298
    View all Publications for this product

    レビューと Q&A

    Show All レビュー Q&A
    レビューを送る 質問を送る

    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.

    Read More
    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.

    Read More

    Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
    For licensing inquiries, please contact partnerships@abcam.com