概述

  • 产品名称Anti-Desmin抗体
    参阅全部 Desmin 一抗
  • 描述
    山羊多克隆抗体to Desmin
  • 经测试应用适用于: WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Chicken, Hamster, Cow, Dog, Pig
  • 免疫原

    Synthetic peptide:

    C-RDGEVVSEATQQQHE

    , corresponding to C-terminal amino acids 454-468 of Human Desmin (NP_001918.3).

  • 阳性对照
    • Human Skeletal Muscle lysate, Human Heart and Human Prostate tissues

性能

相关产品

应用

Our Abpromise guarantee covers the use of ab109980 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
WB Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 55 kDa (predicted molecular weight: 54 kDa).
IHC-P Use a concentration of 3.75 µg/ml.

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 图像

  • Anti-Desmin antibody (ab109980) at 0.2 µg/ml + Human Skeletal Muscle lysate (in RIPA buffer) at 35 µg
    Developed using the ECL technique

    Predicted band size : 54 kDa
  • Human Heart: Formalin-Fixed, Paraffin-Embedded (FFPE) using ab109980 at a concentration of 3.75 µg/ml.
  • Human Prostate: Formalin-Fixed, Paraffin-Embedded (FFPE) using ab109980 at a concentration of 3.75 µg/ml.

Anti-Desmin antibody (ab109980)参考文献

ab109980 has not yet been referenced specifically in any publications.

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