Coagulation Factor X

Coagulation factor X, also known as FX, F10, Eponym Stuart-Prower factor, and thrombokinase, is an enzyme of the coagulation cascade. It is one of the vitamin K-dependent serine proteases, and plays a crucial role in the coagulation cascade and blood clotting, as the first enzyme in the common pathway of thrombus formation. Factor X deficiency is one of the rarest of the inherited coagulation disorders. FX deficiency among the most severe of the rare coagulation defects, typically including hemarthroses, hematomas, and umbilical cord, gastrointestinal, and central nervous system bleeding. Factor X is synthesized in the liver as a mature heterodimer formed from a single-chain precursor, and vitamin K is essential for its synthesis. Factor X is activated into factor Xa (FXa) by both factor IX (with its cofactor, factor VIII in a complex known as intrinsic Xase) and factor VII (with its cofactor, tissue factor in a complex known as extrinsic Xase) through cleaving the activation propeptide. As the first member of the final common pathway or thrombin pathway, FXa converts prothrombin to thrombin in the presence of factor Va, Ca2+, and phospholipid during blood clotting and cleaves prothrombin in two places (an arg-thr and then an arg-ile bond). This process is optimized when factor Xa is complexed with activated cofactor V in the prothrombinase complex. Inborn deficiency of factor X is very uncommon, and may present with epistaxis (nose bleeds), hemarthrosis (bleeding into joints) and gastrointestinal blood loss. Apart from congenital deficiency, low factor X levels may occur occasionally in a number of disease states. Furhermore, factor X deficiency may be seen in amyloidosis, where factor X is adsorbed to the amyloid fibrils in the vasculature.

Coagulation Factor X Related Areas

Enzyme>>Protease & Regulator>>Serine Protease & Regulator>>Coagulation Factor X

Immunology>>Innate Immunity>>Coagulation>>Coagulation Factor X

Cardiovascular>>Coagulation>>Coagulation Factor X

Coagulation Factor X Alternative Names

Coagulation Factor X, FX, F10, FXA, RP11-98F14.9 [Homo sapiens]

Coagulation Factor X, fX, F10, Cf10 [Mus musculus]

Summaries for Coagulation Factor X

Entrez Gene summary for Coagulation Factor X:

This gene encodes the vitamin K-dependent coagulation factor X of the blood coagulation cascade. This factor undergoes multiple processing steps before its preproprotein is converted to a mature two-chain form by the excision of the tripeptide RKR. Two chains of the factor are held together by 1 or more disulfide bonds; the light chain contains 2 EGF-like domains, while the heavy chain contains the catalytic domain which is structurally homologous to those of the other hemostatic serine proteases. The mature factor is activated by the cleavage of the activation peptide by factor IXa (in the intrisic pathway), or by factor VIIa (in the extrinsic pathway). The activated factor then converts prothrombin to thrombin in the presence of factor Va, Ca+2, and phospholipid during blood clotting. Mutations of this gene result in factor X deficiency, a hemorrhagic condition of variable severity.

OMIM - description for Coagulation Factor X:

Factor X deficiency is a rare autosomal recessive bleeding disorder showing variable phenotypic severity. Affected individuals can manifest prolonged nasal and mucosal hemorrhage, menorrhagia, hematuria, and occasionally hemarthrosis. The disorder can be caused either by reduced levels of the factor X protein or by synthesis of a dysfunctional factor X protein

Wikipedia summary for Coagulation Factor X:

Factor X, also known by the eponym Stuart-Prower factor or as prothrombinase, is an enzyme (EC 3.4.21.6) of the coagulation cascade. It is a serine endopeptidase (protease group S1).

Human Coagulation Factor X Protein General Information

 

• Protein names: Recommended name: Coagulation factor XIII A chain Short name=Coagulation factor XIIIa
• Sequence length: 732 AA.
• Sequence similarities: Belongs to the transglutaminase superfamily. Transglutaminase family.
• Post-translational modification: The activation peptide is released by thrombin.
• Catalytic activity: Protein glutamine + alkylamine = protein N(5)-alkylglutamine + NH3.
• Subcellular location: Cytoplasm. Secreted. Note: Secreted into the blood plasma. Cytoplasmic in most tissues, but also secreted in the blood plasma.
• Polymorphism: There are four main allelic forms of this protein; F13A*1A, F13A*1B, F13A*2A and F13A*2B. In addition two other intermediate forms (F13A*2A and F13A*2B) seem to exist. The sequence shown is that of F13A*2B.
• Involvement in disease: Defects in F13A1 are the cause of factor XIII subunit A deficiency (FA13AD) [MIM:613225]. FA13AD is an autosomal recessive disorder characterized by a life-long bleeding tendency, impaired wound healing and spontaneous abortion in affected women.

General information above from UniProt

Function for Coagulation Factor X Protein

UniProtKB:

Factor XIII is activated by thrombin and calcium ion to a transglutaminase that catalyzes the formation of gamma-glutamyl-epsilon-lysine cross-links between fibrin chains, thus stabilizing the fibrin clot. Also cross-link alpha-2-plasmin inhibitor, or fibronectin, to the alpha chains of fibrin.

Genatlas:

• coagulation factor X (Stuart Prower), vitamin K-dependent, converting prothrombin to thrombin in the presence of FVa, Ca2+ and phospholipid

Phenotype Information for Coagulation Factor X

• Gene/Locus: F10
• Phenotype: Factor X deficiency

Phenotype Information for Coagulation Factor X from OMIM (Online Mendelian Inheritance in Man)

Drugs for Coagulation Factor X

Target	Drug Name	Disease	Drug Status
Coagulation Factor X	Enoxaparin	Venous thromboembolism	Approved
Coagulation Factor X	Fondaparinux sodium	Venous thromboembolism	Approved
Coagulation Factor X	Coagulation Factor IX	Christmas disease (hemophilia B)	Approved
Coagulation Factor X	Danaparoid	Deep venous clots	Approved

Drugs for Coagulation Factor X from TTD (Therapeutic Targets Database)