概述

  • 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
  • 特异性ab14452 recognises dystrophin and shows no cross reaction to C protein, a actin, or muscle spectrin.
  • 经测试应用适用于: Other, ELISA, Electron Microscopy, IHC-P, IHC-Frmore details
  • 种属反应性
    与反应: Human
  • 免疫原

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

  • 阳性对照
    • Skeletal muscle.

性能

应用

Our Abpromise guarantee covers the use of ab14452 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
Other
AP
ELISA
Electron Microscopy
IHC-P
IHC-Fr
  • 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.
    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] (ab14452)参考文献

    This product has been referenced in:

    See 1 Publication for this product

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