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Rabbit Anti-Thrombomodulin/FITC Conjugated antibody
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 SLCtype lectin domain.
Contains 6 EGF-like domains.
Database links:
Entrez Gene: 7056 Human
Omim: 37640 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.
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