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Symbol PROC contributors: mct/pgu - updated : 09-09-2014
HGNC name protein C (inactivator of coagulation factors Va and VIIIa)
HGNC id 9451
Corresponding disease
PROC thrombophilia , hereditary, due to protein C deficiency
Location 2q14.3      Physical location : 128.175.995 - 128.186.821
Synonym name
  • anticoagulant protein C
  • blood coagulation factor XIV
  • autoprothrombin IIA
  • protein C
  • vitamin K-dependent protein C
  • activated protein C
  • Synonym symbol(s) PC, PROC1, APC, PC1, THPH3, THPH4
    TYPE functioning gene
    STRUCTURE 10.80 kb     9 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    regulatory sequence Binding site   enhancer   silencer
    motif repetitive sequence   other
    text structure
  • hepatocyte nuclear factor binding sites
  • a strong silencer upstream of the promoter
  • two liver-specific enhancer regions
  • a regulator region in intron 1
  • MAPPING cloned Y linked N status confirmed
    Physical map
    LOC150554 2q21.1 tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein, zeta pseudogene GYPC 2q14-q21 glycophorin C (Gerbich blood group) LOC339760 2q21.1 hypothetical LOC339760 BIN1 2q14 bridging integrator 1 LOC389029 2 LOC389029 LOC339761 2q21.1 similar to 25-hydroxyvitamin D-1 alpha hydroxylase, mitochondrial precursor (Calcidiol 1-monooxygenase) (25-OHD-1 alpha-hydroxylase) (25-hydroxyvitamin D(3) 1-alpha-hydroxylase) (VD3 1A hydroxylase) (P450C1 alpha) (P450VD1-alpha) ERCC3 2q21 excision repair cross-complementing rodent repair deficiency, complementation group 3 (xeroderma pigmentosum group B complementing) MAP3K2 2q14.3 mitogen-activated protein kinase kinase kinase 2 LOC389030 2 LOC389030 PROC 2q13-q14 protein C (inactivator of coagulation factors Va and VIIIa) FLJ10006 2q21.1 hypothetical protein FLJ10006 LOC389031 2 similar to myosin-VIIb MYO7B 2q21.1 myosin VIIB LIMS2 2q14.2 LIM and senescent cell antigen-like domains 2 GPR17 2q21 G protein-coupled receptor 17 MGC5391 2q21.1 hypothetical protein MGC5391 FLJ11294 2q21.1 hypothetical protein FLJ11294 POLR2D 2q21 polymerase (RNA) II (DNA directed) polypeptide D MGC4268 2q21 hypothetical protein MGC4268 SAP130 2q21.1 mSin3A-associated protein 130 UGCGL1 2q14.2 UDP-glucose ceramide glucosyltransferase-like 1 HS6ST1 2q21 heparan sulfate 6-O-sulfotransferase 1 LOC391432 2 similar to hypothetical protein LOC389032 2 LOC389032 LOC391433 2 similar to peptidyl-Pro cis trans isomerase
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    9 - 1790 - 461 - 2007 17646160
    Type restricted
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Reproductivefemale systembreastmammary gland  
    SystemCellPubmedSpeciesStageRna symbol
    Cardiovascularendothelial cell
    cell lineage
    cell lines
    at STAGE
  • two EGF-like domains
  • one Gla (gamma-carboxy-glutamate) domain
  • one peptidase S1 domain
  • conjugated GlycoP
    isoforms Precursor precursor cleaved into a light and a heavy chains held together by a disulfide bond and then activated by thrombin at the surface of endothelial cells
  • peptidase S1 family
  • CATEGORY enzyme
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    text plasma
    basic FUNCTION
  • vitamin K-dependent serine protease regulating blood coagulation by inactivating factors Va and VIIIa in the presence of calcium ions and phospholipids
  • negative regulation of apoptosis and blood coagulation
  • has an important role in governing intestinal microvascular inflammation
  • may protect macro and microcirculation from endotoxic shock
  • inhibits LPS-stimulated complement factor B promoter activity and protein expression, and thus have beneficial effect in therapy in sepsis
  • attenuating both hepatic and renal injury caused by hepatic ischemia and reperfusion injury
  • procoagulant role of PROC pathway may have important implications for the regulation of F3- and TFPI-dependent biologic responses and for fine tuning of the hemostatic balance in the vascular system
  • vitamin K-dependent serine protease zymogen in plasma which upon activation to activated protein C (PROC) by thrombin down-regulates the clotting cascade by limited proteolysis of the procoagulant cofactors Va and VIIIa
  • cytoprotective anticoagulant that can promote cutaneous healing
  • may influence bone cells and has potential to be a therapeutic drug for bone regeneration
  • CELLULAR PROCESS cell life, cell death/apoptosis
    PHYSIOLOGICAL PROCESS coagulation/hemostasis
  • PROC pathway is a unique system involved in controlling intestinal homeostasis and inflammation by regulating epithelial barrier function
  • a component
    small molecule metal binding,
  • Ca2+
  • Zinc ions bind to and inhibit activated protein C
  • protein
  • FVa
  • FVIIIa
  • using the endothelial cell protein C receptor as a coreceptor for cleavage of PAR1
  • ligation of LRP8 causes Dab1-dependent signaling thereby contributing to PROC's beneficial effects on cells
  • induces F2R phosphorylation and desensitizes endothelial cells to thrombin signaling but promotes limited receptor cleavage and negligible internalization and degradation even after prolonged PROC exposure
  • induced endoplasmic reticulum stress and attenuated lipopolysaccharide-induced umbilical vein endothelial cell apoptosis mediated by GSK3B
  • F7 and PROC are phosphatidic acid-binding proteins
  • cell & other
    activated by thrombin, PROS1
    Other vitamin K-dependent
    corresponding disease(s) PROC
    related resource Database of Mutations Protein C
  • to meningococcal disease in young children
  • to variation of bone mineral density in Caucasian men and women
  • Variant & Polymorphism other
  • promoter genotype might significantly influence susceptibility for meningococcal disease in young children
  • CNV significantly associated with bone mineral density in Caucasian men and women
  • Candidate gene
  • PROC pathway is defective in patients with inflammatory Bowel disease
  • Marker
  • decreased PROC levels in patients with type 2 diabetes may serve as a biomarker to indicate the early development of diabetic nephropathy
  • Therapy target
    Zn2+ supports a physiological role in the regulation of anticoagulant activity of PROC and for treatments of thrombotic diseases