PROTEIN C: A MISSING LINK IN THE SUSCEPTIBILITY AND SEVERITY OF SICKLE CELL ANAEMIA

Sickle cell anemia belongs to a group of diseases called sickle cell disease.Sickle cell anemia is an inherited red blood cell disease in which there is not enough healthy red blood cells to transport oxygen throughout the body.Sickle cell anemia is caused by a mutation in a gene (hemoglobin), which is an iron-rich compound that allows red blood cells to carry oxygen from the lungs to the body.In sickle cell anemia, abnormal hemoglobin can cause red blood cells to become stiff, sticky, and deformed.Protein C is a vitamin K-dependent coagulation inhibitor produced in the liver.It works with its cofactor protein S (PS).The activated PC inhibits activated factors V and Viii, thereby reducing the production of thrombin.PC deficiency is associated with an increased risk of thrombotic complications.Protein C, activated by thrombin and in the presence of protein S, inactivates factors V and VIII.Thus, the protein C / S pathway is an effective source of natural anticoagulant activity.Protein C and protein S genetic defect status is associated with an increased risk of venous thromboembolism.In SS disease, both adults and children have been reported to have low levels of protein C and S, which can theoretically lead to a tendency for thrombosis.