Overview

  • Product nameAnti-Dystrophin antibody [0.N.258]
    See all Dystrophin primary antibodies
  • Description
    Mouse monoclonal [0.N.258] to Dystrophin
  • Specificityab14452 recognises dystrophin and shows no cross reaction to C protein, a actin, or muscle spectrin.
  • Tested applicationsSuitable for: Other, ELISA, Electron Microscopy, IHC-P, IHC-Frmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

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

  • Positive control
    • Skeletal muscle.

Properties

Applications

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.

Application Abreviews Notes
Other
AP
ELISA
Electron Microscopy
IHC-P
IHC-Fr
  • Application notesAP: 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.
  • Target

    • FunctionAnchors 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.
    • Tissue specificityExpressed 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.
    • Involvement in diseaseDefects 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.
    • Sequence similaritiesContains 2 CH (calponin-homology) domains.
      Contains 22 spectrin repeats.
      Contains 1 WW domain.
      Contains 1 ZZ-type zinc finger.
    • Cellular localizationCell membrane > sarcolemma. Cytoplasm > cytoskeleton.
    • Information by UniProt
    • Database links
    • Alternative names
      • 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

    References for Anti-Dystrophin antibody [0.N.258] (ab14452)

    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"