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整合素样金属蛋白酶与凝血酶13型抗体

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  • ¥1580 - 2480
  • gelatins
  • jlcR5856
  • 国内
  • 2025年07月07日
  • WB,ELISA等
  • 人/动物/植物
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    • 详细信息
    • 文献和实验
    • 技术资料
    • 供应商

      江西江蓝纯生物试剂有限公司

    • 库存

      183

    • 克隆性

      单克隆

    • 保质期

      1年

    • 抗体英文名

      ADAMTS13

    • 抗体名

      整合素样金属蛋白酶与凝血酶13型抗体

    • 适应物种

      人/动物/植物

    • 应用范围

      WB,ELISA等

    • 浓度

      1mg/ml

    • 保存条件

      -20 °

    • 规格

      100ul/200ul

    规格:100ul产品价格:¥1580.0
    规格:200ul产品价格:¥2480.0
    产品 :  jlcR5856
    英文名称 :  ADAMTS13
    中文名称 :  整合素样金属蛋白酶与凝血酶13型抗体
        :  Cleaves the vWF multimers in plasma into smaller forms. Von Willebrand factor cleaving protease; A disintegrin and metalloproteinase with thrombospondin motifs 13; A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13; A disintegrin like and metalloprotease with thrombospondin type 1 motif 13; ADAM metallopeptidase with thrombospondin type 1 motif 13; ADAM TS 13; ADAM TS13; ADAM-TS 13; ADAM-TS13; ADAMTS 13; ADAMTS-13; ADAMTS13; ADAMTS13 protein; ATS13_HUMAN; C9orf8; TTP; von Willebrand factor-cleaving protease; vWF cleaving protease; vWF CP; vWF-cleaving protease; vWF-CP; vWFCP.  
    研究领域 :  肿瘤  细胞生物  免疫学  信号转导  细胞粘附分子  
    抗体来源 :  Rabbit
    克隆类型 :  Polyclonal
    交叉反应 :   Human, Mouse, Rat, Dog, Pig, Cow, Horse,
    产品应用 :  WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500 (石蜡切片需做抗原修复)
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
     :  145kDa
    细胞定位 :  分泌型蛋白
        :  Lyophilized or Liquid
        :  1mg/ml
     :  KLH conjugated synthetic peptide derived from human ADAMTS13:401-500/1427
        :  IgG
    纯化方法 :  affinity purified by Protein A
     :  0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存条件 :  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    PubMed :  PubMed
    产品介绍 :   This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motif) protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. Individual members of this family differ in the number of C-terminal TS motifs, and some have unique C-terminal domains. The enzyme encoded by this gene is the von Willebrand Factor (vWF)-cleaving protease, which is responsible for cleaving at the site of Tyr842-Met843 of the vWF molecule. A deficiency of this enzyme is associated with thrombotic thrombocytopenic purpura. Alternative splicing of this gene generates multiple transcript variants encoding different isoforms. [provided by RefSeq, Nov 2008].

    Function:
    Cleaves the vWF multimers in plasma into smaller forms.

    Subcellular Location:
    Secreted. Note=Secretion enhanced by O-fucosylation of TSP type-1 repeats.

    Tissue Specificity:
    Plasma. Expressed primarily in liver.

    Post-translational modifications:
    Glycosylated. O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X(2)-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS13. May also be C-glycosylated on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and also N-glycosylated. These other glycosylations can also facilitate secretion.
    The precursor is processed by a furin endopeptidase which cleaves off the pro-domain.

    DISEASE:
    Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP) [MIM:274150]; also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever.

    Similarity:
    Contains 2 CUB domains.
    Contains 1 disintegrin domain.
    Contains 1 peptidase M12B domain.
    Contains 8 TSP type-1 domains.

    SWISS:
    Q76LX8

    Gene ID:
    11093

    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

    细胞外基质蛋白

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