亚洲中文字幕特级毛片-亚洲制服丝袜中文字幕-亚洲制服丝袜在线观看-亚洲制服欧美自拍另类-免费一级黄色-免费一级国产生活片

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
中文字幕亚洲一区二区va在线,丰满熟妇乱又伦在线无码视频,四虎影视永久在线观看
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-VWF/PE-Cy7 Conjugated antibody (bs-0586R-PE-Cy7)
訂購熱線:400-901-9800
訂購郵箱:sales@www.chomd.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@www.chomd.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-0586R-PE-Cy7
英文名稱 Rabbit Anti-VWF/PE-Cy7 Conjugated antibody
中文名稱 PE-Cy7標記的血管假性血友病因子/血管性血友病因子抗體
別    名 Von Willebrand Factor; Coagulation factor VIII; F8VWF; Factor VIII related antigen; von Willebrand antigen 2; Von Willebrand antigen II; Von Willebrand disease; VWD; VWF; VWF_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 心血管  細胞生物  免疫學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat,  (predicted: Dog, Pig, Cow, Rabbit, )
產(chǎn)品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 309kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human von Willebrand antigen 2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
Von Willebrand Factor (VWF) was previously known as Factor VIII related antigen. VWF is synthesized exclusively by endothelial cells and megakaryocytes, and stored in the intracellular granules or constitutively secreted into plasma. This glycoprotein functions as both an antihemophilic factor carrier and a platelet vessel wall mediator in the blood coagulation system. Important in the maintenance of homeostasis, it participates in platelet vessel wall interactions by forming a noncovalent complex with coagulation factor VIII at the site of vascular injury. The Von Willebrand factor has functional binding domains to platelet glycoprotein Ib, glycoprotein IIb/IIIa, collagen and heparin. Mutations in this gene or deficiencies in this protein result in Von Willebrand's disease. VWD is characterized by frequent bleeding (gingival, minor skin quantitative lacerations, menorrhagia, etc.).

Function:
Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma.

Subunit:
Multimeric. Interacts with F8.

Subcellular Location:
Secreted. Secreted, extracellular space, extracellular matrix. Note=Localized to storage granules.

Tissue Specificity:
Plasma.

Post-translational modifications:
All cysteine residues are involved in intrachain or interchain disulfide bonds.
N- and O-glycosylated.

DISEASE:
Defects in VWF are the cause of von Willebrand disease type 1 (VWD1) [MIM:193400]. A common hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 1 is characterized by partial quantitative deficiency of circulating von Willebrand factor, that is otherwise structurally and functionally normal. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma.
Defects in VWF are the cause of von Willebrand disease type 2 (VWD2) [MIM:613554]. A hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 2 is characterized by qualitative deficiency and functional anomalies of von Willebrand factor. It is divided in different subtypes including 2A, 2B, 2M and 2N (Normandy variant). The mutant VWF protein in types 2A, 2B and 2M are defective in their platelet-dependent function, whereas the mutant protein in type 2N is defective in its ability to bind factor VIII. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma.
Defects in VWF are the cause of von Willebrand disease type 3 (VWD3) [MIM:277480]. A severe hemorrhagic disorder due to a total or near total absence of von Willebrand factor in the plasma and cellular compartments, also leading to a profound deficiency of plasmatic factor VIII. Bleeding usually starts in infancy and can include epistaxis, recurrent mucocutaneous bleeding, excessive bleeding after minor trauma, and hemarthroses.

Similarity:
Contains 1 CTCK (C-terminal cystine knot-like) domain.
Contains 4 TIL (trypsin inhibitory-like) domains.
Contains 3 VWFA domains.
Contains 3 VWFC domains.
Contains 4 VWFD domains.

Database links:

Entrez Gene: 280958 Cow

Entrez Gene: 399544 Dog

Entrez Gene: 7450 Human

Entrez Gene: 399543 Pig

Entrez Gene: 116669 Rat

Omim: 613160 Human

SwissProt: Q28295 Dog

SwissProt: P04275 Human

SwissProt: Q28833 Pig

SwissProt: Q62935 Rat

Unigene: 440848 Human

Unigene: 35561 Rat



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

血管性血友病因子(vWF)是血管內(nèi)皮細胞和骨髓巨核細胞合成的一種糖蛋白,在1期和2期止血中都起著重要作用,如缺乏將導致患者出現(xiàn)血管性血友病(vWD)。vWF可被ADAMTS13裂解以失去活性,血小板反應蛋白/凝血酶敏感蛋白-1(Thrombospondin,TSP-1))可參與了這個調(diào)節(jié)過程。vWF水平受多種遺傳和環(huán)境因素影響,其中ABO血型影響較大。vWF主要通過A1和A3區(qū)與血小板GP 1b和膠原結(jié)合,在止血和血栓形成過程中起重要作用,并與心、腦血管疾病及血管新生密切相關(guān),因此研究vWF的生物學特性和功能具有重要的意義。
版權(quán)所有 2004-2026 www.www.chomd.com.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
主站蜘蛛池模板: 成全动漫视频在线观看免费高清版下载亚洲一区二区 | 人人妻人人爽人人澡人人少妇,久... | 日本阿v高清一| 激情无码中文字幕在线观| 96精品久久久无码午| 五月份视频在线播放| 国语精品一区二区三区AV在线观看| 2024国片精品无套内谢无码| 少妇色情影片在线观看男同女 | 玩弄人妻少妇500系列视频0| 国产一区二区精品久久岳,精品精品国产自在 | 一级做a爱片特黄在线观看放学多后| 少妇人妻无码精品视频 熟女| 国内永久免费saas crm| 一级毛片恃级毛片直播,一级毛片完整免费版,亚 | 亚洲五月六月丁香激情 91大神| 奶交app下载| 丰满少妇作爱视频免费观看夫妻隔帘按摩视频 | 丰满少妇人妻无码精品内射| 亚洲AV午夜成人片精品漫日日骚| 精品香蕉在线观看免费网站| 一本色道久久综合一区二区三区,免费人 | 亚洲午夜在线播放91| 精品国产一区二区三区久久久狼,91精品一区二 | 人妻熟妇中文字幕无码老熟妇女| zljzljzlj水多日本| 人妻中文字幕乱人伦在线国语解说视屏 | 国产香蕉在线观看视频在线观看视频 | 激情亚洲欧洲1区,2区,3区| 亚洲熟妇vs亚洲熟妇AV| 国产在线精品区二区不卡99 | 久久综合给合精欧精品欧| 中国护士18XXXXHD少妇| 精品人妻伦一二三区久久1| 亚洲人一二三区深喉视频集锦 | 亚洲精品午夜无码专区_播放灌醉水嫩| 狠狠综合久久久爆操无码综合网浪潮 | 亚洲一本之道高清无码都| 国产亚洲婷婷香蕉久久精品_精品| 亚洲美女群交颜射吞精小说| 国产成人精品电影在线观看81|