Overview

  • Product name
    Anti-Dystrophin antibody, prediluted
    See all Dystrophin primary antibodies
  • Description
    Rabbit polyclonal to Dystrophin, prediluted
  • Host species
    Rabbit
  • Tested applications
    Suitable for: IHC-Fr, IHC-Pmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Rat
  • Positive control
    • Skeletal muscle

Properties

Applications

Our Abpromise guarantee covers the use of ab15278 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-Fr
IHC-P
  • Application notes
    IHC-P: Working dilution, ready-to-use for 10 minutes at RT. Staining of formalin-fixed tissues requires boiling tissue sections in 1mM EDTA, pH 8.0 for 10 minutes followed by cooling at RT for 20 minutes.
    IHC-Fr: Use at an assay dependent dilution (PMID 18384691).


    Not tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 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

    • Immunohistochemical staining of human skeletal muscle with ab15278

    • ab15278 staining Dystrophin in transverse sections of tibialis anterior (TA) muscles of mdx mice by Immunohistochemistry (Frozen sections). Mice were killed and TA muscles were removed, pinned to parafilm-covered cork, snap frozen in liquid N2-cooled isopentane, and stored at -80°C. Sections were cut using cryostat and blocked with 10% normal goat serum in 1% BSA/PBS for 1 h and then incubated with primary antibody for 1 hour. A Cy3 conjugated anti rabbit IgG at 1/500 dilution was used as secondary. Slides were coverslipped with Vectashield mounting medium with DAPI and imaged.

    References

    This product has been referenced in:
    • Praissman JL  et al. The functional O-mannose glycan on a-dystroglycan contains a phospho-ribitol primed for matriglycan addition. Elife 5:N/A (2016). IF . Read more (PubMed: 27130732) »
    • Williams JH  et al. Nanopolymers improve delivery of exon skipping oligonucleotides and concomitant dystrophin expression in skeletal muscle of mdx mice. BMC Biotechnol 8:35 (2008). IHC-Fr ; Mouse . Read more (PubMed: 18384691) »
    See all 2 Publications for this product

    Customer reviews and Q&As

    Answer

    Thank you for your inquiry.


    Based on its primary structure, the dystrophin can be divided into four distinct structural domains: (1) the N-terminal actin-binding domain (aa 14-240), (2) the large triple helical spectrin-like domain (aa 253-3040) composed of 24 repeating units similar to those in β-spectrin, which are predicted to form triple-helical coiled-coils, (3) the cysteine-rich domain (aa 3080- 3360) and the C-terminal domain (aa 3361- 3685). http://www.sciencedirect.com/science/article/pii/0092867488903832


    Please find information on the location of the immunogens by antibody below:


    ab15278: C-terminal

    ab154168: C-terminal

    ab129996: epitope not mapped to our knowledge

    ab14452: epitope not mapped to our knowledge

    ab85302: spectrin-like domain/ rod

    ab7164: C-terminal

    ab15277: C-terminal


    Please note the above antibodies are tested and guaranteed for different species and applications. Please choose carefully. If you let me know what species your samples are from and what application you are planning I will be happy to help choose the most suitable antibody.


    Importantly, I also have to confirm that all our products are for research purposes only and are not intended for diagnostic or therapeutic use.

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

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