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Rabbit Anti-Thrombomodulin/AF647 Conjugated antibody (bs-1783R-AF647)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-1783R-AF647
英文名称 Anti-Thrombomodulin/AF647
中文名称 AF647标记的凝血调节蛋白/血栓调节素抗体
别    名 Thrombomodulin; CD 141; CD141; CD141 antigen; Fetomodulin; THBD; THRM; TM; AHUS 6; AHUS6; BDCA 3; BDCA3; THPH12; TRBM_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 心血管  转运蛋白  糖蛋白  内皮细胞  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat,  (predicted: Dog, Cow, )
产品应用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 58/63kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CD141 N-terminus
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.
产品介绍 background:
Thrombomodulin, TM is cell surface glycoprotein; plays an important role in the protein C anticoagulant pathway. It located in a vein, artery and capillary endothelial cells on the surface of plasma membrane protein. It is generally believed: TM vascular endothelial injury is an important parameter is the thrombin receptor, known in a variety of normal human tissues, can also be expressed in many tumors, TM may be similar to the E-cadherin,and is a lectin Like activity of a new class of members of the cell adhesion molecules.
CD141/Thrombomodulin is an exclusively endothelial cell surface glycoprotein that forms a 1:1 complex with thrombin. Binding of thrombin to this high-affinity receptor alters its specificity toward several substrates. The complex activates protein C approximately 1000 times faster than thrombin alone. Activated protein C degrades clotting factors V and VIII; thus, thrombomodulin converts thrombin into a physiologic anticoagulant. Thrombomodulin is also found in the circulatory and urinary systems, the physiologic significance of this is obscure.

Function:
Thrombomodulin is a specific endothelial cell receptor that forms a 1:1 stoichiometric complex with thrombin. This complex is responsible for the conversion of protein C to the activated protein C (protein Ca). Once evolved, protein Ca scissions the activated cofactors of the coagulation mechanism, factor Va and factor VIIIa, and thereby reduces the amount of thrombin generated.

Subcellular Location:
Membrane; Single-pass type I membrane protein.

Tissue Specificity:
Endothelial cells are unique in synthesizing thrombomodulin.

Post-translational modifications:
N-glycosylated.
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.

DISEASE:
Defects in THBD are the cause of thrombophilia due to thrombomodulin defect (THPH12) [MIM:614486]. A hemostatic disorder characterized by a tendency to thrombosis.
Defects in THBD are a cause of susceptibility to hemolytic uremic syndrome atypical type 6 (AHUS6) [MIM:612926]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype.

Similarity:
Contains 1 C-type lectin domain.
Contains 6 EGF-like domains.

Database links:

Entrez Gene: 7056 Human

Entrez Gene: 83580 Rat

Omim: 188040 Human

SwissProt: P07204 Human

Unigene: 2030 Human

Unigene: 88295 Rat



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

CD141是一种转膜糖蛋白,又称;血栓调节素,表达于许多正常组织中如动、静脉内皮细胞,以及间皮细胞.
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