Overview

  • Product name
    Anti-Dystrophin antibody [MANDRA1]
    See all Dystrophin primary antibodies
  • Description
    Mouse monoclonal [MANDRA1] to Dystrophin
  • Host species
    Mouse
  • Tested applications
    Suitable for: ELISA, IHC-Fr, WB, ICC/IFmore details
    Unsuitable for: IHC-P
  • Species reactivity
    Reacts with: Mouse, Rat, Human, Fish
  • Immunogen

    Fusion protein, corresponding to amino acids 3200-3684 of Human Dystrophin.

  • Epitope
    128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (a.a. residues 3558-3684).
  • Positive control
    • lympho blastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells
  • General notes

    The C-terminal domain of the human dystrophin molecule (a.a. residues 3558-3684) is present in normal muscle tissue. It is also present in nearly all Becker muscular dystrophies, but is absent in cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx).

     

Properties

Applications

Our Abpromise guarantee covers the use of ab7164 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
ELISA Use at an assay dependent concentration.
IHC-Fr Use at an assay dependent concentration.
WB Use at an assay dependent concentration.
ICC/IF Use at an assay dependent concentration. PubMed: 22869749
  • Application notes
    Is unsuitable for IHC-P.
  • 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

    • Anti-Dystrophin antibody [MANDRA1] (ab7164) at 1/300 dilution + whole lysate prepared from mouse myotubes at 30 µg

      Secondary
      Rabbit Anti-Mouse IgG H&L (HRP) (ab6728) at 1/8000 dilution

      Developed using the ECL technique.

      Exposure time: 5 minutes

      See Abreview

    • ab7164 staining Dystrophin in frozen human tongue tissue sections by Immunohistochemistry (IHC - Fr- Frozen sections). Samples were incubated 1:100 dilution. A Goat Anti-mouse, FITC- conjugate was used as the secondary antibody. 

    References

    This product has been referenced in:
    • Baruffaldi F  et al. Dynamic Phosphorylation of the Myocyte Enhancer Factor 2Ca1 Splice Variant Promotes Skeletal Muscle Regeneration and Hypertrophy. Stem Cells 35:725-738 (2017). Read more (PubMed: 27612437) »
    • Sekulic-Jablanovic M  et al. Functional characterization of orbicularis oculi and extraocular muscles. J Gen Physiol 147:395-406 (2016). Read more (PubMed: 27069119) »
    See all 16 Publications for this product

    Customer reviews and Q&As

    1-8 of 8 Abreviews or Q&A

    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.

    Read More

    Question
    Answer

    Thank you for your phone enquiries and interest in our products.

    ab7163 - Anti-Dystrophin antibody [MANDYS8]

    This antibody Monoclonal Anti-Dystrophin, clone MANDYS8, recognizes an epitope located on the rod domain of the human dystrophin molecule, The publications that are cited in the data sheet, which mapped the epitope that the MANDYS8 clone recognises are:

    1. Nguyen, thi Man, et al., FEBS Lett., 262, 237 (1990).
    2. Sedgwick, S., et al. Rapid mapping by transposon mutagenesis of epitopes on the muscular dystrophy protein, dystrophin Nucl. Acids Res. (1991) 19(21): 5889-5894


    According to these references the epitope recognised by this clone lies between residues 1400 and 1505.

    Regarding the epitopes of the other 3 antibodies, ab3149, ab7164, ab15277 - no information is available regarding yet. As soon as we have further details, we will update the corresponding datasheets.

    I hope that this helps and please do not hesitate to contact us if you require any further information or assistance.

    Read More

    Answer

    Thank you for your phone call. As we discussed, this antibody is not conjugated so it should not produce any signal in the absence of a secondary antibody. It is likely that the signal you are observing is autofluorescence. The attached reference details several common types of autofluorescence and tips for avoiding them. I hope this helps, please let me know if you have any additional questions.

    Read More

    Answer

    Thank you for your email. It was nice to speak to you over the phone.
    I am sorry to hear that you are encountering problems with ab7164.

    I am sorry this product did not perform as stated on the datasheet and for the inconvenience this has caused. As requested, I have issued a free of charge replacement with the order number 1017104 with ab3149.

    To check the status of the order please contact our Customer Service team and reference this number.

    Please note that this free of charge replacement vial is also covered by our Abpromise guarantee. Should you still be experiencing difficulties, or if you have any further questions, please do not hesitate to let us know.

    I wish you the best of luck with your research.

    Read More
    Abcam guarantees this product to work in the species/application used in this Abreview.
    Application
    Western blot
    Sample
    Mouse Cell lysate - whole cell (mouse myotubes)
    Loading amount
    30 µg
    Specification
    mouse myotubes
    Gel Running Conditions
    Reduced Denaturing (4-20% gradient gel)
    Blocking step
    BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C

    Abcam user community

    Verified customer

    Submitted Jul 21 2011

    Answer

    Thank you for your e-mail. I would recommend using the same fixative that have been used in the references where staining was done on frozen section; unfortunately I cannot access those papers myself to find this information. I would suspect the fixation will be ice cold acetone or methanol (or a mixture) for 10min, so something similar would be a good starting point. If you have enough cells I would recommend trying in parallel a well with 4%PFA fixing (for 10min) just in case this works in ICC. I hope this will help you, could you please let me know how you get on and do not hesitate to contact me if you need further advice on your staining protocol,

    Read More

    Answer

    Thank you for your enquiry. The antibody will work in frozen sections and I would recommend the following references as sources of staining protocols as they have tested the antibody in IHC: Ellis JM et al. Specificity of dystrophin analysis improved with monoclonal antibodies. Lancet 336:881-2 (1990). PubMed: 1699095. Nguyen TM et al. Monoclonal antibodies for dystrophin analysis. Epitope mapping and improved binding to SDS-treated muscle sections. Biochem J 288 (Pt 2):663-8 (1992). PubMed: 1281410 Gussoni E et al. Normal dystrophin transcripts detected in Duchenne muscular dystrophy patients after myoblast transplantation. Nature 356:435-8 (1992). PubMed: 1557125 I hope these references will help, please let me know if you need further information,

    Read More

    Question
    Answer

    Thank you for your phone call. This clone was tested for application in both direct and indirect ELISA as per the citations below (the PubMed links can be found on the online datasheet for ab7164). Morris GE et al. A quantitative ELISA for dystrophin. J Immunol Methods 161:23-8 (1993). PubMed: 8486926 Nguyen TM et al. Monoclonal antibodies for dystrophin analysis. Epitope mapping and improved binding to SDS-treated muscle sections. Biochem J 288 (Pt 2):663-8 (1992). PubMed: 1281410 These references may offer additional information pertaining to appropriate controls and methodology. If you have any additional questions, please contact us again.

    Read More

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

    Sign up