FGB Human Recombinant Protein

The protein encoded by this gene is the beta component of fibrinogen, a blood-borne glycoprotein comprised of three pairs of nonidentical polypeptide chains. Following vascular injury, fibrinogen is cleaved by thrombin to form fibrin which is the most abundant component of blood clots. In addition, various cleavage products of fibrinogen and fibrin regulate cell adhesion and spreading, display vasoconstrictor and chemotactic activities, and are mitogens for several cell types. Mutations in this gene lead to several disorders, including afibrinogenemia, dysfibrinogenemia, hypodysfibrinogenemia and thrombotic tendency. [provided by RefSeq, Jul 2008]

FGB: Fibrinogen has a double function: yielding monomers that polymerize into fibrin and acting as a cofactor in platelet aggregation. Defects in FGB are a cause of congenital afibrinogenemia (CAFBN). This rare autosomal recessive disorder is characterized by bleeding that varies from mild to severe and by complete absence or extremely low levels of plasma and platelet fibrinogen. Patients with congenital fibrinogen abnormalities can manifest different clinical pictures. Some cases are clinically silent, some show a tendency toward bleeding and some show a predisposition for thrombosis with or without bleeding.

Protein type: Secreted; Cell surface; Adaptor/scaffold; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 4q28

Cellular Component: extracellular space; cell surface; fibrinogen complex; extracellular region; plasma membrane; cell cortex; vesicle; external side of plasma membrane

Molecular Function: protein binding, bridging; protein binding; chaperone binding; cell adhesion molecule binding; structural molecule activity; receptor binding

Biological Process: protein polymerization; platelet activation; extracellular matrix organization and biogenesis; positive regulation of heterotypic cell-cell adhesion; cell-matrix adhesion; signal transduction; platelet degranulation; cellular protein complex assembly; positive regulation of protein secretion; positive regulation of vasoconstriction; innate immune response; response to calcium ion; blood coagulation; positive regulation of exocytosis

Disease: Afibrinogenemia, Congenital; Dysfibrinogenemia, Congenital