概述

  • 产品名称
  • 描述
    小鼠多克隆抗体to LRSAM1
  • 经测试应用
    适用于: ELISA, WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length Human protein

  • 阳性对照
    • Human liver tissue lysate; LRSAM1-transfected 293T cell lysate.

性能

应用

Our Abpromise guarantee covers the use of ab73113 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
ELISA Use at an assay dependent dilution.
WB 1/500 - 1/1000. Predicted molecular weight: 84 kDa.

靶标

  • 功能
    E3 ubiquitin-protein ligase that mediates monoubiquitination of TSG101 at multiple sites, leading to inactivate the ability of TSG101 to sort endocytic (EGF receptors) and exocytic (HIV-1 viral proteins) cargos.
  • 组织特异性
    Highly expressed in adult spinal cord motoneurons as well as in fetal spinal cord and muscle tissue.
  • 通路
    Protein modification; protein ubiquitination.
  • 疾病相关
    Charcot-Marie-Tooth disease 2P (CMT2P) [MIM:614436]: An axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • 序列相似性
    Contains 6 LRR (leucine-rich) repeats.
    Contains 1 RING-type zinc finger.
    Contains 1 SAM (sterile alpha motif) domain.
  • 结构域
    The coiled coil domains interact with the SB domain of TSG101.
    The PTAP motifs mediate the binding to UEV domains.
  • 细胞定位
    Cytoplasm. Displays a punctuate distribution and localizes to a submembranal ring.
  • Information by UniProt
  • 数据库链接
  • 别名
    • CMT2P antibody
    • E3 ubiquitin protein ligase LRSAM1 antibody
    • E3 ubiquitin-protein ligase LRSAM1 antibody
    • EC 6.3.2.- antibody
    • FLJ31641 antibody
    • hTAL antibody
    • Leucine rich repeat and sterile alpha motif containing 1 antibody
    • Leucine rich repeat and sterile alpha motif containing protein 1 antibody
    • Leucine-rich repeat and sterile alpha motif-containing protein 1 antibody
    • LRSAM1 antibody
    • LRSM1_HUMAN antibody
    • OTTHUMP00000022174 antibody
    • OTTHUMP00000022175 antibody
    • RIFLE antibody
    • TAL antibody
    • Tsg101 associated ligase antibody
    • Tsg101-associated ligase antibody
    see all

图片

  • Anti-LRSAM1 antibody (ab73113) at 1/500 dilution + Human liver tissue lysate at 25 µg

    Secondary
    HRP-conjugated Goat Anti-Mouse IgG at 1/2500 dilution

    Predicted band size : 84 kDa
    Observed band size : 84 kDa
  • All lanes : Anti-LRSAM1 antibody (ab73113) at 1/500 dilution

    Lane 1 : LRSAM1-transfected 293T cell lysate
    Lane 2 : Non-transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.

    Secondary
    HRP-conjugated Goat Anti-Mouse IgG at 1/2500 dilution

    Predicted band size : 84 kDa
    Observed band size : 84 kDa

实验方案

文献

This product has been referenced in:
  • Bogdanik LP  et al. Loss of the E3 ubiquitin ligase LRSAM1 sensitizes peripheral axons to degeneration in a mouse model of Charcot-Marie-Tooth disease. Dis Model Mech 6:780-92 (2013). WB . Read more (PubMed: 23519028) »
  • Nicolaou P  et al. A novel LRSAM1 mutation is associated with autosomal dominant axonal Charcot-Marie-Tooth disease. Eur J Hum Genet : (2012). Human . Read more (PubMed: 22781092) »

See all 3 Publications for this product

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