Protein C est un gène codé par le symbole PROC. Communément appelé aussi: Vitamin K-dependent Anticoagulant Autoprothrombin IIA; Blood coagulation factor XIV; PROC. Protein C a une masse de 52.07kDa, une longueur d'acide aminé de 461, et est impliqué dans les maladies: Thrombophilia due to protein C deficiency, autosomal dominant; Thrombophilia due to protein C deficiency, autosomal recessive.
Nous proposons 6 des anticorps contre Protein C, élevé dans Lapin, Souris et Chèvre, qui sont appropriés pour le WB, ELISA et ICC/IF avec des échantillons dérivés de Humain, Souris et Rat.
Informations sur les Gènes et les Protéines
Résumé UniProt
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).
Résumé Entrez
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.
Spécificité tissulaire
Plasma; synthesized in the liver.
Implication dans la maladie
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.
Similitudes de séquence
Belongs to the peptidase S1 family.
Modification post-traductionnelle
The vitamin K-dependent, enzymatic carboxylation of some Glu residues allows the modified protein to bind calcium.
Localisation cellulaire
Secreted. Golgi apparatus. Endoplasmic reticulum.
