InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PROC

Summary

InnateDB Protein

IDBP-69080.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

PROC

Protein Name

protein C (inactivator of coagulation factors Va and VIIIa)

Synonyms

APC; PC; PROC1; THPH3; THPH4;

Species

Homo sapiens

Ensembl Protein

ENSP00000234071

InnateDB Gene

IDBG-69078 (PROC)

Protein Structure

UniProt Annotation

Function

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.

Subcellular Localization

Disease Associations

Thrombophilia due to protein C deficiency, autosomal dominant (THPH3) [MIM:176860]: 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. {ECO:0000269PubMed:1301959, ECO:0000269PubMed:1347706, ECO:0000269PubMed:1511989, ECO:0000269PubMed:1868249, ECO:0000269PubMed:2437584, ECO:0000269PubMed:2602169, ECO:0000269PubMed:7792728, ECO:0000269PubMed:7865674, ECO:0000269PubMed:8292730, ECO:0000269PubMed:8398832, ECO:0000269PubMed:8499568, ECO:0000269PubMed:8560401, ECO:0000269PubMed:8829639, ECO:0000269PubMed:9798967}. Note=The disease is caused by mutations affecting the gene represented in this entry.Thrombophilia due to protein C deficiency, autosomal recessive (THPH4) [MIM:612304]: 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. {ECO:0000269PubMed:1511988, ECO:0000269PubMed:1593215, ECO:0000269PubMed:1611081, ECO:0000269PubMed:7841323, ECO:0000269PubMed:7841324, ECO:0000269PubMed:7878626}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Plasma; synthesized in the liver.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 9 experimentally validated interaction(s) in this database.

Experimentally validated
Total	9 [view]
Protein-Protein	9 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0004252	serine-type endopeptidase activity
GO:0005509	calcium ion binding
GO:0005515	protein binding
GO:0008233	peptidase activity

Biological Process

GO:0006508	proteolysis
GO:0007596	blood coagulation
GO:0017187	peptidyl-glutamic acid carboxylation
GO:0030195	negative regulation of blood coagulation
GO:0043066	negative regulation of apoptotic process
GO:0043687	post-translational protein modification
GO:0044267	cellular protein metabolic process
GO:0050900	leukocyte migration

Cellular Component

GO:0005576	extracellular region
GO:0005788	endoplasmic reticulum lumen
GO:0005796	Golgi lumen
GO:0005886	plasma membrane

Protein Structure and Domains

PDB ID

InterPro

IPR000294 Gamma-carboxyglutamic acid-rich (GLA) domain
IPR000742 Epidermal growth factor-like domain
IPR001254 Peptidase S1
IPR001314 Peptidase S1A, chymotrypsin-type
IPR001881 EGF-like calcium-binding domain
IPR009003 Trypsin-like cysteine/serine peptidase domain
IPR012224 Peptidase S1A, coagulation factor VII/IX/X/C/Z

PFAM

PF00594
PF00008
PF00089
PF07645

PRINTS

PR00001
PR00722

PIRSF

PIRSF001143

SMART

SM00069
SM00181
SM00020
SM00179

TIGRFAMs

Post-translational Modifications

Modification

Cross-References