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CASQ1 Antibody

  • 货号：
    CSB-PA004556LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: CASQ1 antibody at 5µg/ml + Mouse brain tissue
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 46 kDa
      Observed band size: 46 kDa
    • Immunohistochemistry of paraffin-embedded human skeletal muscle tissue using CSB-PA004556LA01HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human gastric cancer using CSB-PA004556LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) CASQ1 Polyclonal antibody
  • Uniprot No.：
    P31415
  • 基因名：
    CASQ1
  • 别名：
    Calmitin antibody; Calmitine antibody; Calsequestrin 1 (fast twitch; skeletal muscle) antibody; Calsequestrin antibody; Calsequestrin; cell antibody; Calsequestrin; skeletal muscle isoform antibody; Calsequestrin-1 antibody; CASQ antibody; Casq1 antibody; CASQ1_HUMAN antibody; PDIB1 antibody; skeletal muscle isoform antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Calsequestrin-1 protein (127-396AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，CASQ1 Antibody (CSB-PA004556LA01HU)，的标记方式是Non-conjugated。对于CASQ1 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-PA004556LB01HU CASQ1 Antibody, HRP conjugated ELISA
    FITC CSB-PA004556LC01HU CASQ1 Antibody, FITC conjugated
    Biotin CSB-PA004556LD01HU CASQ1 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 1:1000-1:5000
    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：
    Calsequestrin is a high-capacity, moderate affinity, calcium-binding protein and thus acts as an internal calcium store in muscle. Calcium ions are bound by clusters of acidic residues at the protein surface, often at the interface between subunits. Can bind around 80 Ca(2+) ions. Regulates the release of lumenal Ca(2+) via the calcium release channel RYR1; this plays an important role in triggering muscle contraction. Negatively regulates store-operated Ca(2+) entry (SOCE) activity.
  • 基因功能参考文献：
    1. These results widen the spectrum of skeletal muscle diseases associated with CASQ1 and indicate that these mutations affect properties critical for correct Ca(2+) handling in skeletal muscle fibers. PMID: 28895244
    2. the p.D244G variant in CASQ1 is associated with a skeletal muscle disease and alters sarcoplasmic calcium release PMID: 27196359
    3. Calsequestrin-1 monomers suppress Store-Operated Ca2+ Entry by interacting with STIM1 and attenuating STIM1 aggregation via its C-terminal amino acid 362-396. PMID: 27185316
    4. the protein aggregate myopathy with benign evolution and muscle inclusions composed of excess CASQ1 due to the D244G heterozygous missense mutation in the CASQ1 gene PMID: 26136523
    5. Equilibrium dialysis and turbidity measurements showed that D244G and, to a lesser extent, M87T partially lose Ca(2+) binding exhibited by wild type calsequestrin 1 at high Ca(2+) concentrations. PMID: 26416891
    6. Missense mutation in CASQ1 gene causes the formation of abnormal sarcoplasmic reticulum (SR) vacuoles containing aggregates of CASQ1 results in altered Ca2+ release, and vacuolar myopathy patients phenotype. PMID: 25116801
    7. There is a significant association between SNP A175G and heat stroke. PMID: 24887214
    8. The sarcoplasmic reticulum calcium content in human type II fibres is primarily determined by the CSQ1 abundance, and in type I fibres, by the combined amounts of both CSQ1 and CSQ2. PMID: 24127619
    9. a direct interaction of dysferlin with Trim72/MG53, AHNAK, cytoplasmic dynein, myomesin-2 and calsequestrin-1, but not with caveolin-3 or dystrophin, is reported. PMID: 23792176
    10. CASQ1 is not a major malignant hyperthermia susceptibility locus in the North American population PMID: 23460944
    11. Downregulation of CSQ-1 in diabetic platelets and impairment of CSQ-1 in normal cells leads to disturbed Ca(2+) release, demonstrating a potential role for CSQ-1 in the regulation of the platelet Ca(2+) release process PMID: 22060633
    12. a mechanism for the observed in vitro and in vivo dynamic high-capacity and low-affinity Ca(2+)-binding activity of calsequestrin PMID: 22337878
    13. Single nucleotide polymorphisms within CASQ1 were genotyped in Amish subjects with type 2 diabetes, glucose intolerance and normals. PMID: 15561962
    14. Noncoding single nucleotide polymorphisms in CASQ1 alter diabetes susceptibility, either by a direct effect on CASQ1 gene expression or perhaps by regulating a nearby gene. PMID: 15561963
    15. CASQ1 polymorphism is not associated with type 2 diabetes PMID: 17681849

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  • 相关疾病：
    Myopathy, vacuolar, with CASQ1 aggregates (VMCQA); Myopathy, tubular aggregate, 1 (TAM1)
  • 亚细胞定位：
    Endoplasmic reticulum. Sarcoplasmic reticulum. Sarcoplasmic reticulum lumen. Sarcoplasmic reticulum membrane; Peripheral membrane protein; Lumenal side. Mitochondrion matrix.
  • 蛋白家族：
    Calsequestrin family
  • 组织特异性：
    Expressed in myoblasts (at protein level).
  • 数据库链接：

    HGNC: 1512

    OMIM: 114250

    KEGG: hsa:844

    STRING: 9606.ENSP00000357057

    UniGene: Hs.632476