Fibrinogen Beta Chain (FGB) ELISA Kit for Human

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. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jun 2014]

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

Chromosomal Location of Human Ortholog: 4q31.3

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

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

Biological Process: adaptive immune response; blood coagulation; cell-matrix adhesion; cellular protein complex assembly; extracellular matrix organization; fibrinolysis; induction of bacterial agglutination; innate immune response; plasminogen activation; platelet degranulation; positive regulation of exocytosis; positive regulation of heterotypic cell-cell adhesion; positive regulation of protein secretion; positive regulation of vasoconstriction; protein polymerization; response to calcium ion; toll-like receptor signaling pathway

Disease: Afibrinogenemia, Congenital; Dysfibrinogenemia, Congenital