概述

  • 产品名称Anti-MTM1抗体
    参阅全部 MTM1 一抗
  • 描述
    兔多克隆抗体to MTM1
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse, Human
  • 免疫原

    Full length Human MTM1 (AAH30779.1; amino acids 1-603).

  • 阳性对照
    • Human liver and mouse liver tissue lysates; HepG2 cell lysate; MTM1 transfected 293T cell lysate

性能

应用

Our Abpromise guarantee covers the use of ab103626 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 1 µg/ml. Predicted molecular weight: 70 kDa.

靶标

  • 功能Lipid phosphatase which dephosphorylates phosphatidylinositol 3-monophosphate (PI3P) and phosphatidylinositol 3,5-bisphosphate (PI(3,5)P2). Has also been shown to dephosphorylate phosphotyrosine- and phosphoserine-containing peptides. Negatively regulates EGFR degradation through regulation of EGFR trafficking from the late endosome to the lysosome. Plays a role in vacuolar formation and morphology. Regulates desmin intermediate filament assembly and architecture. Plays a role in mitochondrial morphology and positioning. Required for skeletal muscle maintenance but not for myogenesis.
  • 疾病相关Defects in MTM1 are the cause of centronuclear myopathy X-linked (XCNM) [MIM:310400]; also known as X-linked myotubular myopathy (XLMTM) or myotubular myopathy type 1 (MTM1). Centronuclear myopathies are congenital muscle disorders characterized by progressive muscular weakness and wasting involving mainly limb girdle, trunk, and neck muscles. It may also affect distal muscles. Weakness may be present during childhood or adolescence or may not become evident until the third decade of life. Ptosis is a frequent clinical feature. The most prominent histopathologic features include high frequency of centrally located nuclei in muscle fibers not secondary to regeneration, radial arrangement of sarcoplasmic strands around the central nuclei, and predominance and hypotrophy of type 1 fibers.
  • 序列相似性Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily.
    Contains 1 GRAM domain.
    Contains 1 myotubularin phosphatase domain.
  • 结构域The GRAM domain mediates binding to PI(3,5)P2 and, with lower affinity, to other phosphoinositides.
  • 细胞定位Cytoplasm. Cell membrane. Cell projection > filopodium. Cell projection > ruffle. Late endosome. Localizes as a dense cytoplasmic network. Also localizes to the plasma membrane, including plasma membrane extensions such as filopodia and ruffles. Predominantly located in the cytoplasm following interaction with MTMR12. Recruited to the late endosome following EGF stimulation.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AF073996 antibody
    • CG2 antibody
    • CNM antibody
    • KIAA4176 antibody
    • mKIAA4176 antibody
    • Mtm antibody
    • Mtm1 antibody
    • MTM1_HUMAN antibody
    • MTMX antibody
    • Myotubular myopathy 1 antibody
    • Myotubularin antibody
    • XLMTM antibody
    see all

Anti-MTM1 antibody 图像

  • Anti-MTM1 antibody (ab103626) at 1 µg/ml + Human liver tissue lysate at 50 µg

    Predicted band size : 70 kDa
  • Anti-MTM1 antibody (ab103626) at 1 µg/ml + Mouse liver tissue lysate at 50 µg

    Predicted band size : 70 kDa
  • Anti-MTM1 antibody (ab103626) at 1 µg/ml + HepG2 cell lysate at 50 µg

    Predicted band size : 70 kDa
  • All lanes : Anti-MTM1 antibody (ab103626) at 1 µg/ml

    Lane 1 : MTM1 transfected 293T cell lysate
    Lane 2 : Non-transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.


    Predicted band size : 70 kDa

Anti-MTM1 antibody (ab103626)参考文献

ab103626 has not yet been referenced specifically in any publications.

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