Protein C ist ein Gen, das durch das Symbol PROC kodiert wird. Im Allgemeinen auch bezeichnet als: Vitamin K-dependent Anticoagulant Autoprothrombin IIA; Blood coagulation factor XIV; PROC. Protein C hat eine Masse von 52.07kDa, eine Aminosäurelänge von 461, und ist an folgenden Krankheiten beteiligt: Thrombophilia due to protein C deficiency, autosomal dominant; Thrombophilia due to protein C deficiency, autosomal recessive.
Wir bieten 6 antikörper gegen Protein C, aufgewachsen in Kaninchen, Maus und Ziege, welche geeignet sind für WB, ELISA and ICC/IF mit Proben abgeleitet von Human, Maus und Ratte.
Gen- und Proteininformationen
UniProt Zusammenfassung
Protein C is a vitamin K-dependent serine protease that regulates blood coagulation by inactivating factors Va and VIIIa in the presence of calcium ions and phospholipids (PubMed:25618265). Exerts a protective effect on the endothelial cell barrier function (PubMed:25651845).
Entrez Zusammenfassung
This gene encodes a vitamin K-dependent plasma glycoprotein. The encoded protein is cleaved to its activated form by the thrombin-thrombomodulin complex. This activated form contains a serine protease domain and functions in degradation of the activated forms of coagulation factors V and VIII. Mutations in this gene have been associated with thrombophilia due to protein C deficiency, neonatal purpura fulminans, and recurrent venous thrombosis.
Gewebespezifität
Plasma; synthesized in the liver.
Rolle bei Krankheiten
Thrombophilia due to protein C deficiency, autosomal dominant: A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. Individuals with decreased amounts of protein C are classically referred to as having type I protein C deficiency and those with normal amounts of a functionally defective protein as having type II deficiency.
Thrombophilia due to protein C deficiency, autosomal recessive: A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. It results in a thrombotic condition that can manifest as a severe neonatal disorder or as a milder disorder with late-onset thrombophilia. The severe form leads to neonatal death through massive neonatal venous thrombosis. Often associated with ecchymotic skin lesions which can turn necrotic called purpura fulminans, this disorder is very rare.
Sequenzähnlichkeiten
Belongs to the peptidase S1 family.
Posttranslationale Modifikation
The vitamin K-dependent, enzymatic carboxylation of some Glu residues allows the modified protein to bind calcium.
Zellort
Secreted. Golgi apparatus. Endoplasmic reticulum.
