Fibrinogen/angiopoietin-like
IPR037579
Definition
This entry includes the alpha, beta and gamma chains of fibrinogen (FGA/B/G), Angiopoietin-1/2 (ANGPT1/2) and similar animal proteins. This entry also includes Fibrinogen-like Protein 1 that is a major immune inhibitory ligand of Lymphocyte-activation gene 3 (LAG-3). Blockade of the FGL1-LAG-3 interaction by monoclonal antibodies was shown to stimulate tumor immunity and is therapeutic against established mouse tumors PMID:30580966. Fibrinogens are cleaved by thrombin to yield fibrins, which polymerize to form the insoluble fibrin clot which blocks sites of vascular injury, preventing further blood loss. The clot is subsequently degraded by plasmin (fibrinolysis). There are three fibrinogens, alpha, beta and gamma PMID:7649481. Thrombin activates fibrinogen to release a fibrinopeptide PMID:2404012. The newly exposed N terminus forms an Glu-Lys isopeptide bond, which is stabilized by factor XIIIA PMID:2143188. Fibrinogen is also required for successful pregnancy, and in knockout mice fatal uterine bleeding occurs around the tenth day of gestation PMID:7649481. Several diseases are a result of mutations in the FGA gene, including congenital afibrinogenemia, resulting in a hemophilia which can be mild to severe PMID:25427968; amyloidosis 8, which causes extensive visceral amyloid deposits, renal amyloidosis and hypertension PMID:8097946 and congenital dysfibrinogenemia PMID:8473507. ANGPT1 binds and activates the tyrosine kinase receptor TEK/TIE2 by inducing its dimerisation and tyrosine phosphorylation. It plays an important role in the regulation of angiogenesis, endothelial cell survival, proliferation, migration, adhesion and cell spreading, reorganization of the actin cytoskeleton, but also maintenance of vascular quiescence [[cite:PMID:18425119], [cite:PMID:30689269], [cite:PMID:18425120], [cite:PMID:15284220]]. ANGPT2 binds to TEK/TIE2, competing for the ANGPT1 binding site, and modulating ANGPT1 signalling [[cite:PMID:15284220], [cite:PMID:19116766], [cite:PMID:19223473], [cite:PMID:9204896]].
12 human proteins with this domain
Each is a reviewed human protein in the Seroatlas serome and a potential autoantibody target; this domain groups them into one antibody-relevant category. Every entry links to its own record.
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