protein c function

The normal adult protein C plasma level ranges from about 70% to 140% of normal plasma, but each laboratory must determine its own laboratory-specific reference range. The […] [16]:2381 Once in active form, APC may or may not remain bound to EPCR, to which it has approximately the same affinity as the protein zymogen. Protein C helps your blood clot normally. Acquired reduction in protein C level occurs because of decreased synthesis or posttranslational modification (e.g., liver disease, vitamin K deficiency, warfarin therapy) or increased turnover (e.g., acute thrombosis, ICF/DIC, sepsis, impaired renal function, postoperative state, adult respiratory distress syndrome, plasma exchange, breast cancer, massive hemorrhage).278 A particularly severe form of acquired protein C deficiency has been reported in association with purpura fulminans and ICF/DIC in individuals with acute meningococcal infections.8,359 In contrast to antithrombin, the antigenic concentrations of vitamin K–dependent plasma proteins, including protein C, are often increased in individuals with nephrotic syndrome.360 In patients with low protein C levels the finding should be confirmed on a subsequent sample after exclusion of acquired causes. Both of these actions down-regulate protein C activation. Ultimately, SAP-C molecules, inserted into opposing lipid vesicles, clip one another through domain swapping, thus bringing the vesicles close enough for fusion. If a fetus is homozygous or compound heterozygous for the deficiency, there may be a presentation of purpura fulminans, severe disseminated intravascular coagulation and simultaneous venous thromboembolism in the womb;[21]:1214 this is very severe and usually fatal. The disease belongs to a group of genetic disorders known as thrombophilias.Protein C deficiency is associated with an increased incidence of venous thromboembolism (relative risk 8–10), whereas no association with arterial thrombotic disease has been found. H - H H H l l Nt I - C H to - C H , Nt - to l l - C - go . Pregnancy and oral contraceptive use can increase plasma protein C levels. We use cookies to help provide and enhance our service and tailor content and ads. [16]:2833, The Gla domain is particularly useful for binding to negatively charged phospholipids for anticoagulation and to EPCR for cytoprotection. The gene's symbol approved by the HUGO Gene Nomenclature Committee is "PROC" from "protein C". If either of these two proteins is absent in murine specimens, the mouse dies from excessive blood-clotting while still in an embryonic state. From: Manual of Pediatric Hematology and Oncology (Fifth Edition), 2011, Lei Shen, Björn Dahlbäck, in Handbook of Proteolytic Enzymes (Third Edition), 2013. Other names for CRP are high-sensitivity C-reactive protein (hs-CRP) and ultra-sensitive C … Thrombin itself may also have an effect on the levels of EPCR. Fetal mice with no protein C develop normally at first, but experience severe bleeding, coagulopathy, deposition of fibrin and necrosis of the liver. Homozygous subjects have a risk 80 times higher. [9]:3162,4 Important proteins that APC up-regulates include Bcl-2, eNOS and IAP. While OspC is highly antigenic and can elicit protective antibody responses, the protective range is narrow (Probert and LeFebvre, 1994; Gilmore et al., 1996; Bockenstedt et al., 1997; Probert et al., 1997; Mathiesen et al., 1998; Rousselle et al., 1998; Gilmore and Mbow, 1999; Mbow et al., 1999; Seinost et al., 1999b; Wallich et al., 2001; Jobe et al., 2003; Scheiblhofer et al., 2003; Brown et al., 2005). PC deficiency is rare in healthy populations, with reported incidence of 0.2%–0.4% of unselected subjects. The protein C plasma concentration (65 nmol/L) is about 100-fold lower than the antithrombin plasma concentration (2.5 µmol/L). 2.1) (Ho and O'Brien, 1971). Protein C helps regulate blood clots and is vital to the process of wound healing. 1lqv: Crystal structure of the Endothelial protein C receptor with phospholipid in the groove in complex with Gla domain of protein C. positive regulation of establishment of endothelial barrier, negative regulation of inflammatory response, regulation of circulating fibrinogen levels, epidermal growth factor (EGF)-like domains, GRCh38: Ensembl release 89: ENSG00000115718, GRCm38: Ensembl release 89: ENSMUSG00000024386, "The nucleotide sequence of the gene for human protein C", "Human recombinant protein C for severe sepsis and septic shock in adult and paediatric patients", "Recombinant human activated protein C for severe sepsis in neonates", "Deficiency of protein C in congenital thrombotic disease", "The structure and evolution of a 461 amino acid human protein C precursor and its messenger RNA, based upon the DNA sequence of cloned human liver cDNAs", "Protein C prevents the coagulopathic and lethal effects of Escherichia coli infusion in the baboon", "Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: prediction of a cofactor to activated protein C", "Drotrecogin alfa (activated): the first FDA-approved treatment for severe sepsis", "ProCMD: a database and 3D web resource for protein C mutants", "A targeted point mutation in thrombomodulin generates viable mice with a prethrombotic state", "Disruption of the endothelial cell protein C receptor gene in mice causes placental thrombosis and early embryonic lethality", "Peptide bond cleavages and loss of functional activity during inactivation of factor Va and factor VaR506Q by activated protein C", "Inhibition of thrombin formation by active site mutated (S360A) activated protein C", "Effects of exogenous recombinant APC in mouse models of ischemia reperfusion injury and of atherosclerosis", "The discovery of activated protein C resistance", "Plasmodium falciparum histones induce endothelial proinflammatory response and barrier dysfunction", "Drotrecogin alfa (activated) for adults with severe sepsis and a low risk of death", "Eosinophil cationic granule proteins impair thrombomodulin function.

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