概述

  • 产品名称Anti-Desmin抗体[RD301]
    参阅全部 Desmin 一抗
  • 描述
    小鼠单克隆抗体[RD301] to Desmin
  • 特异性This antibody reacts exclusively with desmin, which is expressed in smooth and striated muscle cells and their tumors e.g. rhabdomyosarcoma and leiomyosarcoma.
  • 经测试应用适用于: IHC-Fr, WB, ICCmore details
  • 种属反应性
    与反应: Mouse, Rat, Rabbit, Chicken, Hamster, Dog, Human, Pig
    不与反应: Zebrafish
  • 免疫原

    Cytoskeletal desmin extract of chicken gizzard.

性能

应用

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.

应用 Ab评论 说明
IHC-Fr 1/100 - 1/2000. with avidin-biotinylated horseradish peroxidase complex (ABC) as detection reagent.
WB 1/100 - 1/1000.
ICC Use at an assay dependent concentration.

Permeabilised cells

靶标

  • 功能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.
  • 疾病相关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.
  • 序列相似性Belongs to the intermediate filament family.
  • 细胞定位Cytoplasm.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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

Anti-Desmin antibody [RD301] 图像

  • 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

Anti-Desmin antibody [RD301] (ab8976)参考文献

This product has been referenced in:
  • 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 1 Publication for this product

Product Wall

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 ...

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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. ...

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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 ...

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Thank you for your enquiry and I'm sorry to hear that you are experiencing difficulty with ab8976. Can you please provide me with a few additional details: What dilutions of ab8976 have you tried? Do you know that the secondary antibody is working prop...

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