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  4. LRSAM1 Antibody

LRSAM1 Antibody

  • 中文名稱：
    LRSAM1兔多克隆抗體
  • 貨號：
    CSB-PA751010ESR2HU
  • 規格：
    ￥440
  • 圖片：
    • Immunohistochemistry of paraffin-embedded human skin tissue using CSB-PA751010ESR2HU at dilution of 1:100
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) LRSAM1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    LRSAM1
  • 別名：
    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
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human E3 ubiquitin-protein ligase LRSAM1 protein (1-290AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity Purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 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. Bacterial recognition protein that defends the cytoplasm from invasive pathogens. Localizes to several intracellular bacterial pathogens and generates the bacteria-associated ubiquitin signal leading to autophagy-mediated intracellular bacteria degradation (xenophagy).
  • 基因功能參考文獻：
    1. We identified a novel LRSAM1 missense mutation (c.2120C > T, p.Pro707Leu) mapping to the RING domain. The identified missense mutation, as well as of another recently reported pathogenic missense mutation (c.2081G > A, p.Cys694Tyr), revealed that in vitro ubiquitylation activity was largely abrogated. PMID: 28335037
    2. findings suggest that the mutant LRSAM1 may aberrantly affect the formation of transcription machinery. PMID: 27615052
    3. findings demonstrate that the isolated genetic entity Charcot-Marie-Tooth type 2G is caused by a missense mutation in LRSAM1. PMID: 27686364
    4. LRSAM1 exhibited self-association in vitro and in vivo. The study found the self-association of LRSAM1 promotes intermolecular ubiquitination and proved a potential N-terminal ubiquitination. PMID: 28189685
    5. disruption of the C-terminal RING domain confers dominant negative properties to LRSAM1 PMID: 24894446
    6. Our data further confirms that LRSAM1 mutations are associated with CMT2 of AD inheritance. PMID: 22781092
    7. Authors identify LRSAM1 as the E3 ligase responsible for anti-Salmonella autophagy-associated ubiquitination. PMID: 23245322
    8. homozygous mutation in LRSAM1 was proposed as a strong candidate for the disease in a family with recessive axonal polyneuropathy PMID: 22012984
    9. LRSAM1 as a component of the antibacterial autophagic response. PMID: 20616063
    10. LRSAM1 is a strong candidate for the causal gene for the Charcot-Marie-Tooth disease. PMID: 20865121
    11. Results suggest that RIFLE represents a novel signaling protein that mediates components of the Wnt/wingless signaling pathway and cell adhesion in PC12 cells [RIFLE protein]. PMID: 14635195
    12. Tal regulates a Tsg101-associated complex responsible for the sorting of cargo into cytoplasm-containing vesicles that bud at the multivesicular body and at the plasma membrane PMID: 15256501
    13. Tal polyubiquitinates lysine residues in the C-terminus of uncomplexed Tsg101, resulting in proteasomal degradation. PMID: 18077552

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  • 相關疾病：
    Charcot-Marie-Tooth disease 2P (CMT2P)
  • 亞細胞定位：
    Cytoplasm.
  • 組織特異性：
    Highly expressed in adult spinal cord motoneurons as well as in fetal spinal cord and muscle tissue.
  • 數據庫鏈接：

    HGNC: 25135

    OMIM: 610933

    KEGG: hsa:90678

    STRING: 9606.ENSP00000300417

    UniGene: Hs.495188



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