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ADAMTS13 抗体

ADAMTS13 适用: 人 WB, IF, IA 宿主: 小鼠 Monoclonal 20A5 unconjugated
产品编号 ABIN2192078
发货至: 中国
  • 抗原 See all ADAMTS13 抗体
    ADAMTS13 (ADAM Metallopeptidase with Thrombospondin Type 1 Motif, 13 (ADAMTS13))
    适用
    • 69
    • 19
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    宿主
    • 50
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    小鼠
    克隆类型
    • 54
    • 15
    单克隆
    标记
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    This ADAMTS13 antibody is un-conjugated
    应用范围
    • 44
    • 22
    • 13
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    Western Blotting (WB), Immunofluorescence (IF), Immunoassay (IA)
    过滤
    0.2 μm filtered
    克隆位点
    20A5
    Top Product
    Discover our top product ADAMTS13 Primary Antibody
  • 应用备注
    For Western blotting and immunofluorescence dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.
    限制
    仅限研究用
  • 缓冲液
    PBS, containing 0.1 % bovine serum albumin and 0.02 % sodium azide.
    储存液
    Sodium azide
    注意事项
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    储存条件
    4 °C
    储存方法
    Product should be stored at 4 °C. Under recommended storage conditions, product is stable for one year.
    有效期
    12 months
  • 抗原
    ADAMTS13 (ADAM Metallopeptidase with Thrombospondin Type 1 Motif, 13 (ADAMTS13))
    别名
    Adamts-13 (ADAMTS13 产品)
    别名
    ADAMTS13 antibody, ADAM-TS13 antibody, ADAMTS-13 antibody, C9orf8 antibody, VWFCP antibody, vWF-CP antibody, Gm710 antibody, ADAM metallopeptidase with thrombospondin type 1 motif 13 antibody, a disintegrin-like and metallopeptidase (reprolysin type) with thrombospondin type 1 motif, 13 antibody, ADAM metallopeptidase with thrombospondin type 1 motif, 13 antibody, ADAMTS13 antibody, Adamts13 antibody, adamts13 antibody
    背景
    The monoclonal antibody 20A5 recognizes human ADAMTS-13, A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13. ADAMTS-13 is produced by hepatic stellate cells and in smaller amounts by human endothelial cells, and is present in plasma at a concentration of approximately 1 μg/mL. ADAMTS-13 is a zinc-containing metalloprotease belonging to the ADAMTS family characterized by a protease domain, an adjacent disintegrin-like domain, one or more thrombospondin type 1 repeats, a cystein-rich domain and a typical spacer region. ADAMTS-13 is composed of a series of domains (amino to carboxy terminal): metalloprotease, disintegrin-like, central thrombospondin-1 (TSP-1), cysteine-rich, spacer, seven additional TSP-1 domains and two unique CUB domains. ADAMTS-13 has no hydrophobic transmembrane domain, and hence it is not anchored in the cell membrane. The apparent molecular weight is 170 or 190 kDa on non-reducing or reducing SDS-PAGE, respectively. ADAMTS-13 has an important function in haemostasis, where it catalyzes the cleavage of the peptide bond between tyrosine-1605 and methionine-1606 in the A2 domain of von Willebrand Factor (VWF), resulting in 2 electrophoretic reduced fragments of 176 and 140 kDa, respectively. This process renders large multimers less adhesive and hence less reactive in the setting of thrombus formation. ADAMTS-13 is therefore said to be a natural anti-thrombotic agent. Severe ADAMTS-13 deficiency is associated with systemic microvascular thrombosis in familial or acquired thrombotic thrombocytopenic purpura (TTP). The accumulation of non-cleaved large VWF multimers causes spontaneous systemic platelet aggregation blocking oxygen supply to vital organs. This life-threatening disorder can lead to ischemic disease with (multiple) organ failure. The monoclonal antibody 20A5 recognizes the central to C-terminal TSP-1 repeats 2 to 5 of ADAMTS- 13 (amino acid 686-894). Aliases Von Willebrand Factor-Cleaving Protease (VWFCP)
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