Recombinant
RabMAb

Recombinant Anti-Dystrophin antibody [EPR21189] (ab218198)

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Overview

  • Product name

    Anti-Dystrophin antibody [EPR21189]
    See all Dystrophin primary antibodies

  • Description

    Rabbit monoclonal [EPR21189] to Dystrophin

  • Host species

    Rabbit

  • Tested applications

    Suitable for: IHC-P, IHC-Frmore details

  • Species reactivity

    Reacts with: Mouse, Rat

  • Immunogen

    Synthetic peptide within Mouse Dystrophin aa 3650 to the C-terminus. The exact sequence is proprietary.
    Database link: P11531

  • Positive control

    • IHC-P: Mouse cardiac muscle and skeletal muscle tissue; rat colon tissue.

  • General notes

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab218198 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
IHC-P 1/1000. Perform heat mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.
IHC-Fr 1/50.

Positive membrane staining of cardiomyocytes on mouse and Rat heart tissue section (PMID: 26430202).

Target

  • Function

    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.

  • Tissue specificity

    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.

  • Involvement in disease

    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.

  • Sequence similarities

    Contains 2 CH (calponin-homology) domains.
    Contains 22 spectrin repeats.
    Contains 1 WW domain.
    Contains 1 ZZ-type zinc finger.

  • Cellular localization

    Cell 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

    Images

    • Immunohistochemistry (Frozen sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)
      Immunohistochemistry (Frozen sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)

      Immunohistochemical (Frozen sections) analysis of rat heart tissue useing ab218198 (1/50 dilution) to stain Dystrophin. ab150077 AlexaFluor®488 Goat anti-Rabbit (1/500) secondary used. DAPI used as a nuclear counter stain. Heat-mediated antigen retrieval using ab94681 (Tris/EDTA buffer, pH 9.0). Fixative 4% PFA, Permeabilisation with 0.2% Triton X-100.

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)

      Immunohistochemical analysis of paraffin-embedded rat colon tissue labeling Dystrophin with ab218198 at 1/1000 dilution, followed by Goat Anti-Rabbit IgG H&L (HRP) Ready to use. Membranous and cytoplasmic staining in smooth muscle of rat colon (PMID: 18806224) is observed. Counter stained with Hematoxylin.

      Secondary antibody only control: Used PBS instead of primary antibody, secondary antibody is Goat Anti-Rabbit IgG H&L (HRP) Ready to use.

      Perform heat-mediated antigen retrieval using ab93684 (Tris/EDTA buffer, pH 9.0).

    • Immunohistochemistry (Frozen sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)
      Immunohistochemistry (Frozen sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)

      Immunohistochemical (Frozen sections) analysis of mouse heart tissue useing ab218198 (1/50 dilution) to stain Dystrophin. ab150077 AlexaFluor®488 Goat anti-Rabbit (1/500) secondary used. DAPI used as a nuclear counter stain. Heat-mediated antigen retrieval using ab94681 (Tris/EDTA buffer, pH 9.0). Fixative 4% PFA, Permeabilisation with 0.2% Triton X-100.

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)

      Immunohistochemical analysis of paraffin-embedded mouse skeletal muscle tissue labeling Dystrophin with ab218198 at 1/1000 dilution, followed by Goat Anti-Rabbit IgG H&L (HRP) Ready to use. Membranous staining on mouse skeletal muscle (PMID: 24793134) is observed. Counter stained with Hematoxylin.

      Secondary antibody only control: Used PBS instead of primary antibody, secondary antibody is Goat Anti-Rabbit IgG H&L (HRP) Ready to use.

      Perform heat-mediated antigen retrieval using ab93684 (Tris/EDTA buffer, pH 9.0).

       

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dystrophin antibody [EPR21189] (ab218198)

      Immunohistochemical analysis of paraffin-embedded mouse cardiac muscle tissue labeling Dystrophin with ab218198 at 1/1000 dilution, followed by Goat Anti-Rabbit IgG H&L (HRP) Ready to use. Membranous staining on mouse cardiac muscle (PMID: 24793134) is observed. Counter stained with Hematoxylin.

      Secondary antibody only control: Used PBS instead of primary antibody, secondary antibody is Goat Anti-Rabbit IgG H&L (HRP) Ready to use.

      Perform heat-mediated antigen retrieval using ab93684 (Tris/EDTA buffer, pH 9.0).

       

    References

    ab218198 has not yet been referenced specifically in any publications.

    Publishing research using ab218198? Please let us know so that we can cite the reference in this datasheet.

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