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Protein C
The protein C pathway’s key enzyme, activated protein C, provides physiologic antithrombotic activity and exhibits both anti-inflammatory and anti-apoptotic activities. Its actions are related to development of thrombosis and ischemic stroke. The protein C pathway of the coagulation of the blood involves the influences of lipids and lipoproteins and the study of the strong epidemiologic association between hyperlipidemia and hypercoagulability.[1]
Additional recommended knowledge
Role in disease
Protein C deficiency is a rare genetic disorder that predisposes to venous thrombosis and habitual abortion. If homozygous, this presents with a form of disseminated intravascular coagulation in newborns termed purpura fulminans; it is treated by replacing the defective protein C. Activated protein C resistance is the inability of protein C to cleave factors V and/or VIII. This may be hereditary or acquired. The best known and most common hereditary form is Factor V Leiden. Acquired forms occur in the presence of elevated Factor VIII concentrations. Warfarin necrosis is acquired protein C deficiency due to treatment with the vitamin K inhibitor anticoagulant warfarin. In initial stages of action, inhibition of protein C may be stronger than inhibition of the vitamin K-dependent coagulation factors (II, VII, IX and X), leading to paradoxical activation of coagulation and necrosis of skin areas. HDL and the effects of activated protein C (APC) on cells is very important.[3] PharmacologyDrotrecogin alpha(activated) is recombinant activated protein C from Ely Lilly Co, USA. It is used in the treatment of severe sepsis, septic shock and disseminated intravascular coagulation. GeneticsThe PROC gene is located on the second chromosome (2q13-q14).
See also
Notes
Categories: Genes on chromosome 2 | Human proteins | Coagulation system | Peripheral membrane proteins | Angiology | Hematology | EC 3.4.21 |
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Protein_C". A list of authors is available in Wikipedia. |