凝血调节蛋白/血栓调节素抗体

英文名称    Thrombomodulin    

中文名称    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.    

凝血调节蛋白/血栓调节素抗体       

说 明 书    0.1ml  0.2ml      

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, Dog, Cow,     

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 Flow-Cyt=1:100-500 ICC=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.    

分 子 量    58/63kDa    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

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 PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

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.    

凝血调节蛋白/血栓调节素抗体 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.

Gene ID:
7056

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.     

凝血调节蛋白/血栓调节素抗体 

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