Fibrinogen [27C8]

27C8

Fibrin degradation products

Fibrinogen

2 mg/ml Storage

ELISA: Use at an assay dependent dilution. Can be paired for ELISA with 20-272-190053 Mouse monoclonal [1F3] to Fibrinogen . WB: Use at an assay dependent dilution. Use under non-reducing conditions. Can be used as an ELISA pair with 20-272-190053 (capture). Not tested in other applications. Optimal dilutions/concentrations should be determined by the end user. Myeloma: Sp2/0 Fibrinogen is the main protein of blood coagulation system. It is a large protein (MW 340 kDa) and it consists of two identical subunits that contain three polypeptide chains: alpha beta and gamma. All chains are connected with each other by a number of disulfide bonds. Fibrinopeptides A (1-16 amino acids) and B (1-17 amino acids) are released by thrombin from the N-terminal parts of alpha- and beta-chains respectively. In this way fibrinogen is converted into fibrin which by means of polymerization forms a fibrin clot. Fibrinogen clotting underlies pathogenesis of MI thromboembolism and thromboses of arteries and veins since fibrin is the main substrate for thrombus formation. Fibrinogen activation is also involved in pathogenesis of inflammation tumor growth and many other diseases.

yielding monomers that polymerize into fibrin and acting as a cofactor in platelet aggregation.

Secreted.

The alpha chain is not glycosylated.

About one-third of the alpha chains in the molecules in blood were found to be phosphorylated.

Defects in FGA are a cause of congenital afibrinogenemia [MIM:202400]. This is a rare autosomal recessive disorder characterized by bleeding that varies from mild to severe and by complete absence or extremely low levels of plasma and platelet fibrinogen. The majority of cases of afibrinogenemia are due to truncating mutations.

Defects in FGA are a cause of amyloidois type 8 (AMYL8) [MIM:105200]; also known as systemic non-neuropathic amyloidosis or Ostertag-type amyloidosis. AMYL8 is a hereditary generalized amyloidosis due to deposition of apolipoprotein A1 fibrinogen and lysozyme amyloids. Viscera are particularly affected. There is no involvement of the nervous system. Clinical features include renal amyloidosis resulting in nephrotic syndrome arterial hypertension hepatosplenomegaly cholestasis petechial skin rash.