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
  • 特異性The antibody is specific to dystrophin and does not react with alpha-actinin and utrophin, an autosomal homologue of dystrophin, also called dystrophin-related protein (DRP). In Western blots, the antibody also stains the 70-75 kDa protein known as Apo-Dystrophin-1 or Dp71. This is detected in the brain as well as in lymphoblastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells (human hepatoma).
  • アプリケーション適用あり: ELISA, IHC-Fr, WB, ICC/IFmore details
    適用なし: IHC-P
  • 種交差性
    交差種: Mouse, Rat, Human, Fish
  • 免疫原

    Fusion protein, corresponding to amino acids 3200-3684 of Human Dystrophin.

  • 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).

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab7164 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
ELISA Use at an assay dependent concentration.
IHC-Fr Use at an assay dependent concentration.
WB Use at an assay dependent concentration.
ICC/IF Use at an assay dependent concentration. PubMed: 22869749
  • 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

    Anti-Dystrophin antibody [MANDRA1] 画像

    • Anti-Dystrophin antibody [MANDRA1] (ab7164) at 1/300 dilution + whole lysate prepared from mouse myotubes at 30 µg

      Secondary
      Rabbit Anti-Mouse IgG H&L (HRP) (ab6728) at 1/8000 dilution
      Developed using the ECL technique

      Exposure time : 5 minutes

      Image courtesy of an anonymous Abreview.

      See Abreview

    Anti-Dystrophin antibody [MANDRA1] (ab7164) 使用論文

    This product has been referenced in:
    • 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). Read more (PubMed: 23275125) »
    • Wei-Lapierre L  et al. Orai1-dependent calcium entry promotes skeletal muscle growth and limits fatigue. Nat Commun 4:2805 (2013). IHC ; Mouse . Read more (PubMed: 24241282) »

    See all 14 Publications for this product

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    ab7163 - Anti-Dystrophin antibody [MANDYS8]

    This antibody Monoclonal Anti-Dystrophin, clone MANDYS8, recognizes an epitope located on the rod domain of the human dyst...

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    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 detai...

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    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 h...

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    Abcam guarantees this product to work in the species/application used in this Abreview.
    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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    投稿 Jul 21 2011

    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 fi...

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    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 impr...

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    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 dystrophi...

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