製品の概要

  • 製品名Anti-Dystrophin antibody [0.N.258]
    Dystrophin 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal [0.N.258] to Dystrophin
  • 特異性ab14452 recognises dystrophin and shows no cross reaction to C protein, a actin, or muscle spectrin.
  • アプリケーション適用あり: Other, ELISA, Electron Microscopy, IHC-P, IHC-Frmore details
  • 種交差性
    交差種: Human
  • 免疫原

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

  • ポジティブ・コントロール
    • Skeletal muscle.

製品の特性

アプリケーション

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.

アプリケーション Abreviews 特記事項
Other
AP
ELISA
Electron Microscopy
IHC-P
IHC-Fr
  • 追加情報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.
  • 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 [0.N.258] (ab14452) 使用論文

    This product has been referenced in:

    See 1 Publication for this product

    Product Wall

    There are currently no Abreviews or Questions for ab14452.
    Please use the links above to contact us or submit feedback about this product.

    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"