Human Phenotype Ontology

Last uploaded: December 11, 2024
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Preferred Name

Abnormality of the protein C anticoagulant pathway
Synonyms
Definitions

An anomaly of the protein C anticoagulant pathway, which serves as a major system for controlling thrombosis, limiting inflammatory responses, and potentially decreasing endothelial cell apoptosis in response to inflammatory cytokines and ischemia. A natural anticoagulant system denoted the protein C pathway exerts its anticoagulant effect by regulating the activity of FVIIIa and FVa. The vitamin K-dependent protein C is the key component of the pathway. Activated protein C (APC) cleaves and inhibits coagulation cofactors FVIIIa and FVa, which result in downregulation of the activity of the coagulation system. The endothelial protein C receptor stimulates the T-TM-mediated activation of protein C on the endothelial cell surface. The two cofactors, protein S and the intact form of FV, enhance the anticoagulant activity of APC.
ID

http://purl.obolibrary.org/obo/HP_0030780
database_cross_reference

UMLS:C4280774
definition

An anomaly of the protein C anticoagulant pathway, which serves as a major system for controlling thrombosis, limiting inflammatory responses, and potentially decreasing endothelial cell apoptosis in response to inflammatory cytokines and ischemia. A natural anticoagulant system denoted the protein C pathway exerts its anticoagulant effect by regulating the activity of FVIIIa and FVa. The vitamin K-dependent protein C is the key component of the pathway. Activated protein C (APC) cleaves and inhibits coagulation cofactors FVIIIa and FVa, which result in downregulation of the activity of the coagulation system. The endothelial protein C receptor stimulates the T-TM-mediated activation of protein C on the endothelial cell surface. The two cofactors, protein S and the intact form of FV, enhance the anticoagulant activity of APC.
has_obo_namespace

human_phenotype
id

HP:0030780
label

Abnormality of the protein C anticoagulant pathway
notation

HP:0030780
prefLabel

Abnormality of the protein C anticoagulant pathway
treeView

http://purl.obolibrary.org/obo/HP_0003256
subClassOf

http://purl.obolibrary.org/obo/HP_0003256
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