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A2ML1 Antibody

  • 货号：
    CSB-PA001005LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: A549 whole cell lysate
      All lanes: A2ML1 antibody at 3.2µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 162, 106 kDa
      Observed band size: 162 kDa
    • Immunohistochemistry of paraffin-embedded human lung cancer using CSB-PA001005LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品描述：

    The A2ML1 antibody is generated from the rabbit immunized with a partial recombinant protein corresponding to 625-751 amino acids of human A2ML1 protein. It exhibits the unconjugated IgG isoform. Its specificity for human A2ML1 protein has been validated in ELISA, IHC, and WB applications. The purity of this A2ML1 antibody is greater than 95% using protein G purified.

    Its target protein A2ML1 mainly inhibits protease activity, contributing to protecting tissues from degradation by enzymes. A2ML1 also plays a role in several biological processes, including immune response, wound healing, and tissue regeneration. Mutations in the A2ML1 gene have been associated with several developmental disorders, including Noonan syndrome-like disorder with loose anagen hair.

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) A2ML1 Polyclonal antibody
  • Uniprot No.：
    A8K2U0
  • 基因名：
    A2ML1
  • 别名：
    A2ML1 antibody; CPAMD9Alpha-2-macroglobulin-like protein 1 antibody; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 9 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Alpha-2-macroglobulin-like protein 1 protein (625-751AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，A2ML1 Antibody (CSB-PA001005LA01HU)，的标记方式是Non-conjugated。对于A2ML1 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA001005LB01HU A2ML1 Antibody, HRP conjugated ELISA
    FITC CSB-PA001005LC01HU A2ML1 Antibody, FITC conjugated
    Biotin CSB-PA001005LD01HU A2ML1 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    Is able to inhibit all four classes of proteinases by a unique 'trapping' mechanism. This protein has a peptide stretch, called the 'bait region' which contains specific cleavage sites for different proteinases. When a proteinase cleaves the bait region, a conformational change is induced in the protein which traps the proteinase. The entrapped enzyme remains active against low molecular weight substrates (activity against high molecular weight substrates is greatly reduced). Following cleavage in the bait region a thioester bond is hydrolyzed and mediates the covalent binding of the protein to the proteinase. Displays inhibitory activity against chymotrypsin, papain, thermolysin, subtilisin A and, to a lesser extent, elastase but not trypsin. May play an important role during desquamation by inhibiting extracellular proteases.
  • 基因功能参考文献：
    1. The functional assessment supported the pathogenicity of the RAF1 and RIT1 variants of unknown significance (VUSs), while the significance of two VUSs in A2ML1 remained unclear. PMID: 29402968
    2. The indigenous Filipino population has a ~50% prevalence of otitis media. A2ML1 genotype is the primary risk factor for otitis media and main determinant of disease progression, although age, the middle ear microbiome, and social clusters might modulate the effect of the A2ML1 genotype. PMID: 27484237
    3. A2ML1-related otitis media susceptibility may be mediated by changes in the middle ear microbiome. PMID: 27799062
    4. Studies support a role for alpha-2-macroglobulin-like 1 protein (A2ML1) in the pathophysiology of otitis media. PMID: 26121085
    5. our results provide evidence that mutations in A2ML1 are a cause of Noonan-like syndrome, with a variable phenotype ranging from severe to very mild. PMID: 24939586
    6. a new class of target antigens in a paraneoplastic autoimmune multiorgan syndrome PMID: 20805888
    7. alpha2ML1 is the first alpha2macroglobulin family member detected in the epidermis, where it may play an important role during desquamation by inhibiting extracellular proteases. PMID: 16298998
    8. alpha2ML1 binding to the low density lipoprotein receptor-related protein 1 (LRP1) reveals a new role for LRP1 in the human epidermis PMID: 18648652

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  • 亚细胞定位：
    Secreted.
  • 蛋白家族：
    Protease inhibitor I39 (alpha-2-macroglobulin) family
  • 组织特异性：
    In the epidermis, expressed predominantly in the granular layer at the apical edge of keratinocytes (at protein level). Also detected in placenta, testis and thymus but not in epithelia of kidney, lung, small intestine or colon.
  • 数据库链接：

    HGNC: 23336

    OMIM: 610627

    KEGG: hsa:144568

    STRING: 9606.ENSP00000299698

    UniGene: Hs.620532