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ADAMTS13整合素样金属蛋白酶与凝血酶13型抗体

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  • ¥1380 - 2200
  • LMAI Bio
  • LM-5856R
  • 进口/国产
  • 2025年12月09日
  • 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)
  • Rabbit
  • Human, Mouse, Rat, Dog, Pig, Cow, Horse,
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    • 详细信息
    • 文献和实验
    • 技术资料
    • 供应商

      上海联迈生物工程有限公司

    • 库存

      大量

    • 目录编号

      LM-5856R

    • 克隆性

      多克隆

    • 抗原来源

      Rabbit

    • 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

    • 宿主

      Rabbit

    • 适应物种

      Human, Mouse, Rat, Dog, Pig, Cow, Horse,

    • 免疫原

      KLH conjugated synthetic peptide derived from human ADAMTS13:401-500/1427

    • 亚型

      IgG

    • 形态

      Lyophilized or Liquid

    • 应用范围

      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)

    • 浓度

      1mg/ml

    • 保存条件

      Store at -20 °C

    • 规格

      100ul  200ul

    ADAMTS13整合素样金属蛋白酶与凝血酶13型抗体
    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
    别    名 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.  
    规格价格 100ul/1380元 购买    200ul/2200元 购买    大包装/询价
    说 明 书 100ul  200ul
    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.
    分 子 量 145kDa
    细胞定位 分泌型蛋白 
    性    状 Lyophilized or Liquid
    浓    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human ADAMTS13:401-500/1427 
    亚    型 IgG
    纯化方法 affinity purified by Protein A
    储 存 液 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 background:
    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

    Database links:

    Entrez Gene: 11093 Human

    Entrez Gene: 279028 Mouse

    Entrez Gene: 362091 Rat

    Omim: 604134 Human

    SwissProt: Q76LX8 Human

    SwissProt: Q769J6 Mouse

    Unigene: 131433 Human

    Unigene: 330084 Mouse



    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
    产品图片 产品细节图片1
    Sample: 
    Liver (Mouse) Lysate at 40 ug
    Primary: Anti-ADAMTS13 (bs-5856R) at 1/1000 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 145 kD
    Observed band size: 155 kD

    产品细节图片2
    Tissue/cell: human lung carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded; 
    Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; 
    Incubation: Anti-ADAMTS13 Polyclonal Antibody, Unconjugated(bs-5856R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining

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