Coagulation Factor Viia (F7) Human Active Protein

This gene encodes coagulation factor VII which is a vitamin K-dependent factor essential for hemostasis. This factor circulates in the blood in a zymogen form, and is converted to an active form by either factor IXa, factor Xa, factor XIIa, or thrombin by minor proteolysis. Upon activation of the factor VII, a heavy chain containing a catalytic domain and a light chain containing 2 EGF-like domains are generated, and two chains are held together by a disulfide bond. In the presence of factor III and calcium ions, the activated factor then further activates the coagulation cascade by converting factor IX to factor IXa and/or factor X to factor Xa. Defects in this gene can cause coagulopathy. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, May 2012]

F7: Initiates the extrinsic pathway of blood coagulation. Serine protease that circulates in the blood in a zymogen form. Factor VII is converted to factor VIIa by factor Xa, factor XIIa, factor IXa, or thrombin by minor proteolysis. In the presence of tissue factor and calcium ions, factor VIIa then converts factor X to factor Xa by limited proteolysis. Factor VIIa will also convert factor IX to factor IXa in the presence of tissue factor and calcium. Defects in F7 are the cause of factor VII deficiency (FA7D). A hemorrhagic disease with variable presentation. The clinical picture can be very severe, with the early occurrence of intracerebral hemorrhages or repeated hemarthroses, or, in contrast, moderate with cutaneous-mucosal hemorrhages (epistaxis, menorrhagia) or hemorrhages provoked by a surgical intervention. Finally, numerous subjects are completely asymptomatic despite very low factor VII levels. Belongs to the peptidase S1 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Motility/polarity/chemotaxis; Apoptosis; Secreted; Protease; EC 3.4.21.21

Chromosomal Location of Human Ortholog: 13q34

Cellular Component: extracellular space; endoplasmic reticulum lumen; Golgi lumen; extracellular region; plasma membrane; vesicle

Molecular Function: protein binding; serine-type peptidase activity; serine-type endopeptidase activity; calcium ion binding; glycoprotein binding; receptor binding

Biological Process: circadian rhythm; organ regeneration; positive regulation of positive chemotaxis; positive regulation of blood coagulation; positive regulation of leukocyte chemotaxis; proteolysis; post-translational protein modification; peptidyl-glutamic acid carboxylation; positive regulation of protein kinase B signaling cascade; blood coagulation, extrinsic pathway; cellular protein metabolic process; response to vitamin K; response to estrogen stimulus; blood coagulation; positive regulation of cell migration

Disease: Myocardial Infarction, Susceptibility To; Factor Vii Deficiency