Recombinant human ADAMTS13 protein,N-His (重组蛋白) | Bioss

一键复制 说明书
Recombinant human ADAMTS13 protein,N-His (重组蛋白) | Bioss
货号：bs-42478P
产品详情
相关标记
相关产品
相关文献
常见问题

概述

产品编号
bs-42478P
英文名称
Recombinant human ADAMTS13 protein,N-His
中文名称
重组人整合素样金属蛋白酶与凝血酶13型蛋白
英文别名
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.
性状
Liquid
纯化方法
AC
理论分子量
43 kDa
浓度
>1mg/ml
储存液
20mM Tris-HCL (pH=8.0) with 8M Urea
研究领域

Cancer > Invasion/microenvironment > ECM > Extracellular matrix > ADAM protein family

Cell Biology > Proteolysis / Ubiquitin > Proteolytic enzymes > Metalloprotease > ADAM TS

Signal Transduction > Cytoskeleton / ECM > Extracellular Matrix > ECM Enzymes > ADAM Protein Family

SWISS
Q76LX8
Gene ID
11093
序列
75-441/1427
物种
Human
标签
N-His
纯度
>90% as determined by SDS-PAGE
内毒素
Not analyzed
表达系统
E.coli
复溶
Centrifuge tubes before opening. Reconstituting to a concentration more than 100 μg/ml is recommended. Dissolve the lyophilized protein in distilled water.
保存条件
Stored at -70℃ or -20℃. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
数据库链接

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

产品介绍

细胞外基质蛋白

背景资料

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].

重组人整合素样金属蛋白酶与凝血酶13型蛋白-bs-42478P

相关产品

暂无相关产品

靶标

基因名
ADAMTS13
蛋白名
A disintegrin and metalloproteinase with thrombospondin motifs 13
亚细胞定位
Secreted. Note=Secretion enhanced by O-fucosylation of TSP type-1 repeats.
组织特异性
Plasma. Expressed primarily in liver.
翻译后修饰
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.
疾病
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.
相似性
Contains 2 CUB domains.
Contains 1 disintegrin domain.
Contains 1 peptidase M12B domain.
Contains 8 TSP type-1 domains.
功能
Cleaves the vWF multimers in plasma into smaller forms.

同靶标产品

相关文献

常见问题