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CHRNG Antibody

  • 货号：
    CSB-PA005401ESR2HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: CHRNG antibody at 5.5μg/ml
      Lane 1: 293T whole cell lysate
      Lane 2: HepG2 whole cell lysate
      Lane 3: Hela whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 58, 53 kDa
      Observed band size: 58 kDa
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) CHRNG Polyclonal antibody
  • Uniprot No.：
    P07510
  • 基因名：
    CHRNG
  • 别名：
    Acetylcholine receptor muscle gamma subunit antibody; Acetylcholine receptor protein gamma chain precursor antibody; Acetylcholine receptor subunit gamma antibody; ACHG antibody; ACHG_HUMAN antibody; Achr 3 antibody; AChR antibody; Achr3 antibody; ACHRG antibody; ACRG antibody; Cholinergic receptor nicotinic gamma antibody; Cholinergic receptor nicotinic gamma polypeptide antibody; CHRNG antibody; MGC133376 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Acetylcholine receptor subunit gamma protein (328-517AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:1000-1:5000
  • 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：
    After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane.
  • 基因功能参考文献：
    1. CHRNG mutations associated with interfamilial variability of the severity of the Escobar variant of multiple pterygium syndrome PMID: 27245440
    2. Two siblings with Escobar syndrome caused by homozygous mutations of the CHRNG gene were identified. PMID: 25411939
    3. Rare cases of congenital arthrogryposis multiplex caused by novel recurrent CHRNG mutations. PMID: 25608830
    4. We did not identify a clear difference in mutation spectrum of the CHRNG gene between lethal form and non-lethal forms of multiple pterygium syndromes PMID: 22167768
    5. CHRNG mutations identified in families with Escobar syndrome show that the trait is a congenital dysmorphology caused by transient inactivation of the neuromuscular endplate. PMID: 16826520
    6. Mutations cxause lethal and nonlethal forms of multiple pterygium syndrome. PMID: 16826531
    7. constructed and characterized four AChR gamma extracellular domain variants PMID: 18502212
    8. This study suggests for the first time in humans, a possible role for genetic variation in the neuromuscular nicotinic acetylcholine receptor, particularly the gamma subunit, in systolic blood pressure regulation PMID: 18625075
    9. Detection of PAX3/7-FKHR fusion gene by one-step RT-PCR is useful in the diagnosis of rhabdomyosarcomas (RMS) and that AChR-gamma is overexpressed in Chinese RMS patients. PMID: 18988640

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  • 相关疾病：
    Multiple pterygium syndrome, lethal type (LMPS); Multiple pterygium syndrome, Escobar variant (EVMPS)
  • 亚细胞定位：
    Cell junction, synapse, postsynaptic cell membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Ligand-gated ion channel (TC 1.A.9) family, Acetylcholine receptor (TC 1.A.9.1) subfamily, Gamma/CHRNG sub-subfamily
  • 数据库链接：

    HGNC: 1967

    OMIM: 100730

    KEGG: hsa:1146

    STRING: 9606.ENSP00000374145

    UniGene: Hs.248101