protein

NATIVE HUMAN FIBRINOGEN

MBS230141

25 mg

230 USD

Purified Protein

NATIVE HUMAN FIBRINOGEN

FIBRINOGEN

fibrinogen alpha chain isoform alpha-E preproprotein; Fibrinogen alpha chain; fibrinogen alpha chain; fibrinogen, A alpha polypeptide; fibrinogen alpha chain

FGA; FGA; Fib2

FIBA_HUMAN

866

35-65% protein, of which >90% clottable protein.Virtually free from plasminogen and plasmin.

Purified. Purified protein from human plasma - liquid

Approximate Protein Concentration: 1.25 mg/ml fibrinogen

Store at -20 degree C only. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the protein. Should this product contain a precipitate we recommend microcentrifugation before use. Shelf Life: 12 months from date of despatch.

ELISA (EIA), Functional Assays (FN)

Preparation: Protein purified by precipitation

Buffer Solution: Citrate buffered saline. Target Species: Human

Fibronectin is a 340kDa glycoprotein involved in cell adhesion and migration processes including embryogenesis, metastasis, wound healing, blood coagulation and host immunity. It consists of a dimer, each part made up of three disulphide linked polypeptides known as the alpha, beta and gamma chains.

4503689

NP_000499.1

NM_000508.3

340 kD

Amyloids Pathway (366238); Blood Clotting Cascade Pathway (198840); Common Pathway (106060); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Disease Pathway (530764); Extracellular Matrix Organization Pathway (576262); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057); GRB2:SOS Provides Linkage To MAPK Signaling For Integrins Pathway (106055); Hemostasis Pathway (106028)

The protein encoded by this gene is the alpha 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 dysfibrinogenemia, hypofibrinogenemia, afibrinogenemia and renal amyloidosis. Alternative splicing results in two isoforms which vary in the carboxy-terminus. [provided by RefSeq, Jul 2008]

FGA: Fibrinogen has a double function: yielding monomers that polymerize into fibrin and acting as a cofactor in platelet aggregation. Defects in FGA are a cause of congenital afibrinogenemia (CAFBN). 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. Variations in position Arg-35 (the site of cleavage of fibrinopeptide a by thrombin) leads to alpha- dysfibrinogenemias. Defects in FGA are a cause of amyloidosis type 8 (AMYL8); 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. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 4q28. Cellular Component: extracellular space; cell surface; fibrinogen complex; plasma membrane; extracellular region; cell cortex; vesicle; external side of plasma membrane. Molecular Function: protein binding, bridging; protein 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: Amyloidosis, Familial Visceral; Afibrinogenemia, Congenital; Dysfibrinogenemia, Congenital

25 mg

230 USD