Anti-Dystrophin抗体[0.N.258] (ab14451)


  • 产品名称Anti-Dystrophin抗体[0.N.258]
    参阅全部 Dystrophin 一抗
  • 描述
    小鼠单克隆抗体[0.N.258] to Dystrophin
  • 经测试应用适用于: Electron Microscopy, ELISA, ICC/IF, IHC-Fr, Other, IHC-Pmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Xenopus laevis
  • 免疫原

    Acetylcholine receptor (AChR) enriched membranes from Torpedo nobiliana electric organ.



Our Abpromise guarantee covers the use of ab14451 in the following tested applications.

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

应用 Ab评论 说明
Electron Microscopy
  • 应用说明AP: Use at an assay dependent dilution.
    Electron Microscopy: Use at an assay dependent dilution.
    ELISA: Use at an assay dependent dilution.
    IF: Use at an assay dependent dilution.
    IHC-P: Use at a concentration of 2 - 4 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. Boil tissue sections in 1mM EDTA, pH 8.0, for 10-20 min followed by cooling at RT for 20 min. Note that 1mM EDTA, pH 8.0 is better than 10mM citrate buffer, pH 6.0 for unmasking the epitope.
    IHC-Fr: Use at a concentration of 2 - 4 µg/ml.

    Not tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 功能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.
    • 组织特异性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.
    • 疾病相关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.
    • 序列相似性Contains 2 CH (calponin-homology) domains.
      Contains 22 spectrin repeats.
      Contains 1 WW domain.
      Contains 1 ZZ-type zinc finger.
    • 细胞定位Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
    • Information by UniProt
    • 数据库链接
    • 别名
      • 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 [0.N.258] (ab14451)参考文献

    ab14451 has not yet been referenced specifically in any publications.

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    Perdona por la espera. Estaba esperando una contestación por parte de mis compañeros.

    Lamentablemente no hemos podido calcular el porcentaje de homología de ninguno de los anticuerpos anti Dystrophin con la proteín...

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    The immunogen for ab14451 was "Acetylcholine receptor (AChR)-enriched membranes and peripheral membrane proteins from Torpedo nobiliana electric organ." The monoclonal epitope has not been determined. Please do not hesitate to contact us again if ...

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    I have received further information from the source of these antibodies, sorry about the delay. We have currently four antibodies against mouse dystrophin which have been used in ELISA, two of which are not purified (they come from ascitic fluid), ...

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