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  4. SGCB Antibody, FITC conjugated

SGCB Antibody, FITC conjugated

  • 中文名稱：
    SGCB兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA618082LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) SGCB Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    SGCB
  • 別名：
    43 kDa dystrophin associated glycoprotein antibody; 43 kDa dystrophin-associated glycoprotein antibody; 43DAG antibody; A3b antibody; Beta SG antibody; Beta-sarcoglycan antibody; Beta-SG antibody; dystrophin-associated glycoprotein, 43-kD antibody; LGMD2E antibody; Limb girdle muscular dystrophy 2E (non linked families) antibody; Sarcoglycan beta antibody; sarcoglycan, beta (43kDa dystrophin-associated glycoprotein) antibody; SGC antibody; SGCB antibody; SGCB_HUMAN antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Beta-sarcoglycan protein (124-219AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 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
  • 產品提供形式：
    Liquid
  • 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：
    Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.
  • 基因功能參考文獻：
    1. This study demonstrated that LGMD2E is the most common type of sarcoglycanopathies in the Iranian population. PMID: 28687063
    2. Clinical severity of limb-girdle muscular dystrophy type 2Emay be predicted by SGCB gene mutation and sarcoglycan protein expression. PMID: 25862795
    3. Defective assembly of sarcoglycan complex in patients with beta-sarcoglycan gene mutations PMID: 12060343
    4. beta-sarcoglycan and SPATA18 may have a role in limb-girdle muscular dystrophy type 2E PMID: 16088906
    5. While the quantity of beta-sarcoglycan was nearly normal in the limb girdle muscular dystrophy (LGMD)2E carrier, the levels of dysferlin protein were reduced to 50% of controls in the carriers of LGMD2B. PMID: 16934466
    6. These data suggest that formation of the beta-delta-core may promote the export and deposition of sarcoglycan subcomplexes at the plasma membrane, and therefore identifies a mechanism for sarcoglycan transport. PMID: 17036316
    7. The limb-girdle muscular dystrophy patients with beta-sarcoglycan deficient LGMD2E do not enable an accurate prediction of the genotype. PMID: 18996010

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  • 相關疾病：
    Limb-girdle muscular dystrophy 2E (LGMD2E)
  • 亞細胞定位：
    Cell membrane, sarcolemma; Single-pass type II membrane protein. Cytoplasm, cytoskeleton.
  • 蛋白家族：
    Sarcoglycan beta/delta/gamma/zeta family
  • 組織特異性：
    Highest expression in heart and skeletal muscle. Low expression in brain, kidney, placenta, pancreas and lung. High expression in fetal brain. Also found in fetal lung, kidney and liver.
  • 數據庫鏈接：

    HGNC: 10806

    OMIM: 600900

    KEGG: hsa:6443

    STRING: 9606.ENSP00000370839

    UniGene: Hs.438953



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