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F11 / FXI / Factor XI is coagulation factor XI of the blood coagulation cascade. This protein is present in plasma as a zymogen, which is a unique plasma coagulation enzyme because it exists as a homodimer consisting of two identical polypeptide chains linked by disulfide bonds. During activation of the plasma factor XI, an internal peptide bond is cleaved by factor XIIa (or XII) in each of the two chains, resulting in activated factor XIa, a serine protease composed of two heavy and two light chains held together by disulfide bonds. This activated plasma factor XI triggers the middle phase of the intrisic pathway of blood coagulation by activating factor IX. Defects in this factor lead to Rosenthal syndrome, a blood coagulation abnormality.
Gene Name: | coagulation factor XI |
Family/Subfamily: | Protease , Serine S1 |
Synonyms: | F11, Coagulation factor XI, Factor xi deficiency, F11 deficiency, Factor XI, PTA deficiency, PTA, Rosenthal syndrome, FXI |
Target Sequences: | NM_000128 NP_000119.1 P03951 |
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