概述

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

    Recombinant fragment: MASASTSKYN SHSLENESIK RTSRDGVNRD LTEAVPRLPG ETLITDKEVI YICPFNGPIK GRVYITNYRL YLRSLETDSS LILDVPLGVI SRIEKMGGAT , corresponding to amino acids 1-100 of Human MTM1

性能

应用

Our Abpromise guarantee covers the use of ab55532 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-P
WB
  • 应用说明WB: Use at a concentration of 1-5 µg/ml.
    IHC-P: Use at a concentration of 3 µg/ml.

    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 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

    • MTM1 antibody (ab55532) used in immunohistochemistry at 3ug/ml on formalin fixed and paraffin embedded human pancreas.
    • Western blot against tagged recombinant protein immunogen using ab55532 MTM1 antibody at 1ug/ml. Predicted band size of immunogen is 34 kDa

    Anti-MTM1 antibody (ab55532)参考文献

    ab55532 has not yet been referenced specifically in any publications.

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