Endothelial protein C receptor

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"EPCR" redirects here. For the European rugby governing body, see European Professional Club Rugby.
Protein C receptor, endothelial
Protein PROCR PDB 1l8j.png
PDB rendering based on 1l8j.
Available structures
PDB Ortholog search: PDBe, RCSB
External IDs OMIM600646 MGI104596 HomoloGene4670 ChEMBL: 5392 GeneCards: PROCR Gene
RNA expression pattern
PBB GE PROCR 203650 at tn.png
More reference expression data
Species Human Mouse
Entrez 10544 19124
Ensembl ENSG00000101000 ENSMUSG00000027611
UniProt Q9UNN8 Q64695
RefSeq (mRNA) NM_006404 NM_011171
RefSeq (protein) NP_006395 NP_035301
Location (UCSC) Chr 20:
33.76 – 33.77 Mb
Chr 2:
155.75 – 155.76 Mb
PubMed search [1] [2]

Endothelial protein C receptor (EPCR) also known as activated protein C receptor (APC receptor) is a protein that in humans is encoded by the PROCR gene.[1][2][3] PROCR has also recently been designated CD201 (cluster of differentiation 201).

The protein encoded by this gene is a receptor for activated protein C, a serine protease activated by and involved in the blood coagulation pathway. The encoded protein is an N-glycosylated type I membrane protein that enhances the activation of protein C.[3]

Clinical significance[edit]

Mutations in this gene have been associated with venous thromboembolism and myocardial infarction, as well as with late fetal loss during pregnancy.[3]

The protein is also involved in Plasmodium falciparum malaria as subtypes of the P. falciparum erythrocyte membrane protein 1 (PfEMP1) family use EPCR of the host as a receptor.[4]


  1. ^ Fukudome K, Esmon CT (Nov 1994). "Identification, cloning, and regulation of a novel endothelial cell protein C/activated protein C receptor". J Biol Chem 269 (42): 26486–91. PMID 7929370. 
  2. ^ Rothbarth K, Dabaghian AR, Stammer H, Werner D (Oct 1999). "One single mRNA encodes the centrosomal protein CCD41 and the endothelial cell protein C receptor (EPCR)". FEBS Lett 458 (1): 77–80. doi:10.1016/S0014-5793(99)01074-1. PMID 10518938. 
  3. ^ a b c "Entrez Gene: PROCR protein C receptor, endothelial (EPCR)". 
  4. ^ Louise Turner and others (2013). "Severe malaria is associated with parasite binding to endothelial protein C receptor". Nature. doi:10.1038/nature12216. 

Further reading[edit]

  • Esmon CT (2004). "Coagulation and inflammation.". J. Endotoxin Res. 9 (3): 192–8. doi:10.1179/096805103125001603. PMID 12831462. 
  • Ruf W, Dorfleutner A, Riewald M (2003). "Specificity of coagulation factor signaling.". J. Thromb. Haemost. 1 (7): 1495–503. doi:10.1046/j.1538-7836.2003.00300.x. PMID 12871285. 
  • Van de Wouwer M, Collen D, Conway EM (2005). "Thrombomodulin-protein C-EPCR system: integrated to regulate coagulation and inflammation.". Arterioscler. Thromb. Vasc. Biol. 24 (8): 1374–83. doi:10.1161/01.ATV.0000134298.25489.92. PMID 15178554. 
  • Medina P, Navarro S, Estellés A, España F (2007). "Polymorphisms in the endothelial protein C receptor gene and thrombophilia.". Thromb. Haemost. 98 (3): 564–9. doi:10.1160/th07-01-0071. PMID 17849044. 
  • Raheja KK (1977). "Nursing in transition.". Nursing forum 15 (4): 413–7. doi:10.1111/j.1744-6198.1976.tb01065.x. PMID 1051893. 
  • Laszik Z, Mitro A, Taylor FB et al. (1998). "Human protein C receptor is present primarily on endothelium of large blood vessels: implications for the control of the protein C pathway.". Circulation 96 (10): 3633–40. doi:10.1161/01.cir.96.10.3633. PMID 9396465. 
  • Ye X, Fukudome K, Tsuneyoshi N et al. (1999). "The endothelial cell protein C receptor (EPCR) functions as a primary receptor for protein C activation on endothelial cells in arteries, veins, and capillaries.". Biochem. Biophys. Res. Commun. 259 (3): 671–7. doi:10.1006/bbrc.1999.0846. PMID 10364477. 
  • Simmonds RE, Lane DA (1999). "Structural and functional implications of the intron/exon organization of the human endothelial cell protein C/activated protein C receptor (EPCR) gene: comparison with the structure of CD1/major histocompatibility complex alpha1 and alpha2 domains.". Blood 94 (2): 632–41. PMID 10397730. 
  • Hayashi T, Nakamura H, Okada A et al. (1999). "Organization and chromosomal localization of the human endothelial protein C receptor gene.". Gene 238 (2): 367–73. doi:10.1016/S0378-1119(99)00360-1. PMID 10570964. 
  • Liaw PC, Neuenschwander PF, Smirnov MD, Esmon CT (2000). "Mechanisms by which soluble endothelial cell protein C receptor modulates protein C and activated protein C function.". J. Biol. Chem. 275 (8): 5447–52. doi:10.1074/jbc.275.8.5447. PMID 10681521. 
  • Xu J, Qu D, Esmon NL, Esmon CT (2000). "Metalloproteolytic release of endothelial cell protein C receptor.". J. Biol. Chem. 275 (8): 6038–44. doi:10.1074/jbc.275.8.6038. PMID 10681599. 
  • Liaw PC, Mather T, Oganesyan N et al. (2001). "Identification of the protein C/activated protein C binding sites on the endothelial cell protein C receptor. Implications for a novel mode of ligand recognition by a major histocompatibility complex class 1-type receptor.". J. Biol. Chem. 276 (11): 8364–70. doi:10.1074/jbc.M010572200. PMID 11099506. 
  • Biguzzi E, Merati G, Liaw PC et al. (2002). "A 23bp insertion in the endothelial protein C receptor (EPCR) gene impairs EPCR function.". Thromb. Haemost. 86 (4): 945–8. PMID 11686350. 
  • Galligan L, Livingstone W, Volkov Y et al. (2001). "Characterization of protein C receptor expression in monocytes.". Br. J. Haematol. 115 (2): 408–14. doi:10.1046/j.1365-2141.2001.03187.x. PMID 11703343. 
  • von Depka M, Czwalinna A, Eisert R et al. (2002). "Prevalence of a 23bp insertion in exon 3 of the endothelial cell protein C receptor gene in venous thrombophilia.". Thromb. Haemost. 86 (6): 1360–2. PMID 11776299. 
  • Deloukas P, Matthews LH, Ashurst J et al. (2002). "The DNA sequence and comparative analysis of human chromosome 20.". Nature 414 (6866): 865–71. doi:10.1038/414865a. PMID 11780052. 
  • Oganesyan V, Oganesyan N, Terzyan S et al. (2002). "The crystal structure of the endothelial protein C receptor and a bound phospholipid.". J. Biol. Chem. 277 (28): 24851–4. doi:10.1074/jbc.C200163200. PMID 12034704. 
  • Riewald M, Petrovan RJ, Donner A et al. (2002). "Activation of endothelial cell protease activated receptor 1 by the protein C pathway.". Science 296 (5574): 1880–2. doi:10.1126/science.1071699. PMID 12052963. 

This article incorporates text from the United States National Library of Medicine, which is in the public domain.