製品の概要

  • 製品名Anti-Dystrophin antibody [SPM499]
    Dystrophin 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal [SPM499] to Dystrophin
  • アプリケーション適用あり: IHC-Pmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Rat
  • 免疫原

    Synthetic peptide, corresponding to C-terminus of Human Dystrophin.

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

法規制情報

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab119698 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
IHC-P 1/50. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

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 [SPM499] 画像

  • Human skeletal muscle tissue stained with ab119698 at a dilution of 1/50.

Anti-Dystrophin antibody [SPM499] (ab119698) 使用論文

ab119698 has not yet been referenced specifically in any publications.

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