Protein C è un gene codificato dal simbolo PROC. Comunemente indicato anche come: Vitamin K-dependent Anticoagulant Autoprothrombin IIA; Blood coagulation factor XIV; PROC. Protein C ha una massa di 52.07kDa, una lunghezza di amminoacidi di 461, ed è implicato nella malattia: Thrombophilia due to protein C deficiency, autosomal dominant; Thrombophilia due to protein C deficiency, autosomal recessive.
Offriamo 6 anticorpi contro Protein C, allevati nel Coniglio, Topo e Capra, che sono adatti per WB, ELISA e ICC/IF con campioni derivati da Umano, Topo e Ratto.
Informazioni su geni e proteine
Riepilogo 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).
Sommario di 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.
Specificità del tessuto
Plasma; synthesized in the liver.
Coinvolgimento nella malattia
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.
Somiglianze di sequenza
Belongs to the peptidase S1 family.
Modifica post-translazionale
The vitamin K-dependent, enzymatic carboxylation of some Glu residues allows the modified protein to bind calcium.
Posizione cellulare
Secreted. Golgi apparatus. Endoplasmic reticulum.
