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CASQ1 Antibody

  • 中文名稱：
    CASQ1兔多克隆抗體
  • 貨號(hào)：
    CSB-PA004556LA01HU
  • 規(guī)格：
    ￥440
  • 圖片：
    • 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：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) CASQ1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    CASQ1
  • 別名：
    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
  • 反應(yīng)種屬：
    Human, Mouse
  • 免疫原：
    Recombinant Human Calsequestrin-1 protein (127-396AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated

    本頁(yè)面中的產(chǎn)品，CASQ1 Antibody (CSB-PA004556LA01HU)，的標(biāo)記方式是Non-conjugated。對(duì)于CASQ1 Antibody，我們還提供其他標(biāo)記。見(jiàn)下表：

    可提供標(biāo)記
    標(biāo)記方式 貨號(hào) 產(chǎn)品名稱 應(yīng)用
    HRP CSB-PA004556LB01HU CASQ1 Antibody, HRP conjugated ELISA
    FITC CSB-PA004556LC01HU CASQ1 Antibody, FITC conjugated
    Biotin CSB-PA004556LD01HU CASQ1 Antibody, Biotin conjugated ELISA
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, WB, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    IHC 1:20-1:200
  • Protocols：
  • 儲(chǔ)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(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 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.
  • 基因功能參考文獻(xiàn)：
    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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  • 相關(guān)疾?。?/div>
    Myopathy, vacuolar, with CASQ1 aggregates (VMCQA); Myopathy, tubular aggregate, 1 (TAM1)
  • 亞細(xì)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
  • 組織特異性：
    Expressed in myoblasts (at protein level).
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 1512

    OMIM: 114250

    KEGG: hsa:844

    STRING: 9606.ENSP00000357057

    UniGene: Hs.632476