整合素样金属蛋白酶与凝血酶13型抗体
规格：1mg/1ml
英文名： ADAMTS13
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 metalloprotea
分子量： 145kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ADAMTS13
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse,
细胞定位：分泌型蛋白
整合素样金属蛋白酶与凝血酶13型抗体产品介绍：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整合素样金属蛋白酶与凝血酶13型抗体 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, 整合素样金属蛋白酶与凝血酶13型抗体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 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
产整合素样金属蛋白酶与凝血酶13型抗体品应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：肿瘤 细胞生物 **学 信号转导 细胞粘附分子
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid