PROS1 : protein S

Description

The PROS1 (protein S) is a protein-coding gene located on chromosome 3.

The PROS1 gene provides instructions for making a protein called protein S, which plays a crucial role in regulating blood clotting. Although protein S can independently inhibit certain clotting factors, it often works as a cofactor, enhancing the function of enzymes that accelerate chemical reactions. Protein S is produced by liver cells and circulates in the bloodstream in two forms: bound to a specific protein or free. The free form of protein S acts as a cofactor for activated protein C (APC), an enzyme that inactivates the clotting proteins factor Va and factor VIIIa. Protein S also aids tissue factor pathway inhibitor (TFPI) in blocking the activity of factor Xa, another clotting protein. This inactivation process slows down clotting, ensuring unimpeded blood flow.

PROS1 is also known as PROS, PS21, PS22, PS23, PS24, PS25, PSA, THPH5, THPH6.

Associated Diseases

• Thrombophilia due to protein S deficiency, autosomal recessive
• Severe hereditary thrombophilia due to congenital protein S deficiency
• Thrombophilia, hereditary, due to protein S deficiency, autosomal
• Protein S deficiency