Information AboutFactor Xi |
| CATEGORIES ABOUT FACTOR XI | |
| ec 3.4.21 | |
| coagulation system | |
| genes associated with genetic disorders | |
Factor XI or '''plasma thromboplastin antecent''' is one of the Enzyme s of the Coagulation Cascade . Like many other coagulation factors, it is a Serine Protease . PHYSIOLOGY Factor XI (FXI) is produced by the Liver and circulates as a homo-dimer in its inactive form. The plasma half-life of FXI is approximately 52 hours. The zymogen factor is activated into ''factor XIa'' by Factor XII a (FXIIa), Thrombin , and it is also Autocatalytic , and FXI is a member of the "contact pathway" due to activation by FXIIa (with includes HMWK , Prekallikrein , Factor XII , factor XI and Factor IX ). Factor XIa activates factor IX by selectively cleaving Arg - Ala and Arg - Val Peptide Bond s. Factor IXa, in turn, activates Factor X . Inhibitors of factor XIa include Protein Z-dependent Protease Inhibitor (ZPI, a member of the Serine Protease Inhibitor /serpin class of proteins), which is independent of Protein Z (its action on factor X, however, is protein Z-dependent, hence its name). PROTEIN STRUCTURE AND MOLECULAR BIOLOGY Although synthesized as a single polypeptide chain, FXI circulates as a homo-dimer. Every chain has a relative molecular mass of approximately 80000. Typical plasma concentrations of FXI are 5 mg/l, corresponding to a plasma concentration (of FXI dimers) of approximately 30nM. The FXI gene is 23kb in length, has 15 exons, and is found on chromosome 4q32-35. ROLE IN DISEASE Deficiency of factor XI causes the rare Haemophilia C ; this mainly occurs in Ashkenazi Jew s and is believed to affect approximately 8% of that population. The condition has been described in other populations at around 1% of cases. It is an Autosomal Recessive disorder. There is little spontaneous bleeding, but surgical procedures may cause excessive blood loss, and prophylaxis is required. Low levels of factor XI also occur in many other disease states, including Noonan Syndrome . High levels of factor XI have been implicated in Thrombosis , although it is uncertain what determines these levels and how serious the procoagulant state is. EXTERNAL LINKS |
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