整合素样金属蛋白酶与凝血酶13型抗体

英文名称  Anti-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. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg    

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat, Dog, Pig, Cow, Horse  

产品类型  一抗    

整合素样金属蛋白酶与凝血酶13型抗体研究领域  肿瘤 细胞生物 免疫学 信号转导 细胞粘附分子  

蛋白分子量  predicted molecular weight: 145kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human ADAMTS13 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  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.  

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. 

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

整合素样金属蛋白酶与凝血酶13型抗体产品介绍 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. 

Database links : UniProtKB/Swiss-Prot: Q76LX8.1