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Rabbit Anti-Thrombomodulin/RBITC Conjugated antibody (bs-0525R-RBITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-0525R-RBITC
英文名稱 Rabbit Anti-Thrombomodulin/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標記的血栓調節(jié)蛋白抗體
別    名 Thrombomodulin; CD 141; CD141; CD141 antigen; Fetomodulin; THBD; THRM; TM; AHUS 6; AHUS6; BDCA 3; BDCA3; THPH12; TRBM_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞生物  神經生物學  血管內皮細胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,  (predicted: Mouse, Rat, Dog, Cow, )
產品應用 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 61kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from mouse Thrombomodulin
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.
產品介紹 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

Omim: 188040 Human

SwissProt: P07204 Human

Unigene: 2030 Human



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

血栓調節(jié)蛋白(thrombomodulin,TM)是一種分布于靜脈、動脈和毛細血管內皮細胞表面的質膜蛋白。 一般認為:TM是血管內皮損傷的重要參數(shù),也是凝血酶的受體,已知在人類多種正常組織中表達,亦可表達于許多腫瘤組織,TM可能類似于鈣粘蛋白,是具有凝集素樣活性的新一類細胞粘附分子的成員。TM是血管內皮細胞膜上的凝血酶受體之一。與凝血酶結合后可降低凝血酶的凝血活性,而加強其激活蛋白C的活性。由于被激活的蛋白C具有抗凝作用,因此,TM是使凝血酶由促凝轉向抗凝的重要的血管內凝血抑制因子。
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