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Rabbit Anti-Protein S/FITC Conjugated antibody
background:
Protein S (PROS) is a vitamin K-dependent plasma protein that inhibits blood clotting by serving as a cofactor for activated protein C (APC) and facilitates clearance of early apoptotic cells. In the plasma, circulating Protein S becomes inactive upon complexing with C4b-binding protein (C4BP); 60-70% of Protein S circulates in complex with C4BP. Calcium-dependent association of C4BP-Protein S with apoptotic cells influences the regulation of complement activation. Protein S has APSLCindependent anticoagulant activity through direct inhibition of prothrombin activation via interactions with Factor X A, Factor V A and phospholipids. Autosomal dominant Protein S deficiency (levels 15 to 37% of normal) correlates with severe recurrent venous thrombosis.
Function:
Anticoagulant plasma protein; it is a cofactor to activated protein C in the degradation of coagulation factors Va and VIIIa. It helps to prevent coagulation and stimulating fibrinolysis.
Subcellular Location:
Secreted
Tissue Specificity:
Plasma.
Post-translational modifications:
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains (By similarity).
DISEASE:
Defects in PROS1 are the cause of thrombophilia due to protein S deficiency, autosomal dominant (THPH5) [MIM:612336]. A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. However, many adults with heterozygous disease may be asymptomatic. Based on the plasma levels of total and free PROS1 antigen as well as the serine protease-activated protein C cofactor activity, three types of PROS1D have been described: type I, characterized by reduced total and free PROS1 antigen levels together with reduced anticoagulant activity; type III, in which only free PROS1 antigen and PROS1 activity levels are reduced; and the rare type II which is characterized by normal concentrations of both total and free PROS1 antigen, but low cofactor activity.
Defects in PROS1 are the cause of thrombophilia due to protein S deficiency, autosomal recessive (THPH6) [MIM:614514]. A very rare and severe hematologic disorder resulting in thrombosis and secondary hemorrhage usually beginning in early infancy. Some affected individuals develop neonatal purpura fulminans, multifocal thrombosis, or intracranial hemorrhage.
Similarity:
Contains 4 EGF-like domains.
Contains 1 Gla (gamma-carboxy-glutamate) domain.
Contains 2 laminin G-like domains.
Database links:
Entrez Gene: 5627 Human
Omim: 176176 Human
SwissProt: P07225 Human
Unigene: 12816 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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