Overview

  • Product name
    Anti-Desmin antibody [RD301]
    See all Desmin primary antibodies
  • Description
    Mouse monoclonal [RD301] to Desmin
  • Host species
    Mouse
  • Specificity
    This antibody reacts exclusively with desmin, which is expressed in smooth and striated muscle cells and their tumors e.g. rhabdomyosarcoma and leiomyosarcoma.
  • Tested applications
    Suitable for: ICC/IF, IHC-Fr, WBmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Rabbit, Chicken, Hamster, Dog, Human, Pig
    Does not react with: Zebrafish
  • Immunogen

    Cytoskeletal desmin extract of chicken gizzard.

Properties

Applications

Our Abpromise guarantee covers the use of ab8976 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
ICC/IF Use at an assay dependent concentration.

Permeabilised cells

 

IHC-Fr 1/100 - 1/2000. with avidin-biotinylated horseradish peroxidase complex (ABC) as detection reagent.
WB 1/100 - 1/1000.

Target

  • Function
    Desmin are class-III intermediate filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures.
  • Involvement in disease
    Defects in DES are the cause of myopathy myofibrillar desmin-related (MFM-DES) [MIM:601419]; also known as desmin-related myopathy (DRM). A neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells.
    Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [MIM:604765]. 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.
    Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400]. Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin.
  • Sequence similarities
    Belongs to the intermediate filament family.
  • Cellular localization
    Cytoplasm.
  • Information by UniProt
  • Database links
  • Alternative names
    • CMD1I antibody
    • CSM1 antibody
    • CSM2 antibody
    • DES antibody
    • DESM_HUMAN antibody
    • Desmin antibody
    • FLJ12025 antibody
    • FLJ39719 antibody
    • FLJ41013 antibody
    • FLJ41793 antibody
    • Intermediate filament protein antibody
    • OTTHUMP00000064865 antibody
    see all

Images

  • Fluorescence microscopy of (a) cultured BHK-21 cells, (b) human skeletal muscle and (c) humanmyocardium stained with the monoclonal desmin antibody ab8976.

  • Immunofluorescence staining of a 7 days old zebrafish embryo

References

This product has been referenced in:
  • Ji HP  et al. MicroRNA-28 potentially regulates the photoreceptor lineage commitment of Müller glia-derived progenitors. Sci Rep 7:11374 (2017). Read more (PubMed: 28900179) »
  • Maiellaro-Rafferty K  et al. Altered regional cardiac wall mechanics are associated with differential cardiomyocyte calcium handling due to nebulette mutations in preclinical inherited dilated cardiomyopathy. J Mol Cell Cardiol 60:151-60 (2013). WB ; Mouse . Read more (PubMed: 23632046) »
See all 2 Publications for this product

Customer reviews and Q&As

1-4 of 4 Abreviews or Q&A

Answer

Thank you for clarifying this information. As you have had good results with the other lot I believe the problems is with the vial you received and I would be happy to offer you a replacement vial if the antibody was purchased in the last 120 days. If you could please provide your order details I can arrange a free order for you, I look forward to hearing from you,

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Answer

Thank you for contacting us for technical support regarding your recent problem with a vial of ab8976. I'm sorry to hear you are experiencing problems with this vial and I would appreciate if you could please clarify which vial is not working for you. According to your orders you have received two tubes: lot 256477 is the most recent one (the one you said worked well) was ordered a month ago lot 225987 was ordered in December 2006 (the one you said did not work). Is this correct or is it the opposite happening i.e. the new vial is not working? Thank you very much for clarifying, I look forward to hearing from you to resolve this matter,

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Answer

Thank you for your email. I would expect for you to detect desmin in your samples, smooth muscle cells, and you certainly have tried high enough concentrations of ab8976. How long did you incubate with the primary? I would suggest incubating overnight at 4C. Other than that, your protocol looks fine to me. We have not had any other complaints regarding this product, and there is a possibilty that the vial you received had gone off during the shipping process. I can offer to send you a free of charge replacement vial of ab8976 or can offer you a credit note or refund. Please let me know which you would prefer.

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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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