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  4. CASQ1 Recombinant Monoclonal Antibody

CASQ1 Recombinant Monoclonal Antibody

  • 中文名稱：
    CASQ1 Recombinant Monoclonal Antibody
  • 貨號：
    CSB-RA166562A0HU
  • 規格：
    ￥1320
  • 圖片：
    • IHC image of CSB-RA166562A0HU diluted at 1:100 and staining in paraffin-embedded human skeletal muscle tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • Overlay Peak curve showing Raji cells stained with CSB-RA166562A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for?10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
    CASQ1
  • 別名：
    Calsequestrin-1 (Calmitine) (Calsequestrin, skeletal muscle isoform), CASQ1, CASQ
  • 反應種屬：
    Human
  • 免疫原：
    A synthesized peptide derived from Human CASQ1 protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Rabbit IgG
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    19D7
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, IHC, FC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:50-1:200
    FC 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：
    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：
    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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  • 相關疾病：
    Myopathy, vacuolar, with CASQ1 aggregates (VMCQA); Myopathy, tubular aggregate, 1 (TAM1)
  • 亞細胞定位：
    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).
  • 數據庫鏈接：

    HGNC: 1512

    OMIM: 114250

    KEGG: hsa:844

    STRING: 9606.ENSP00000357057

    UniGene: Hs.632476



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