Endothelial protein C receptor

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View/Edit Human

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 protein C that enhances its activation. Protein C is an anti-coagulant serine protease activated by the blood coagulation pathway.

Structure

EPCR protein is an N-glycosylated type I membrane protein that enhances the activation of protein C.^[3] It belongs to the MHC class I/CD1 family of proteins, that is characterized by having a deep groove, that in other proteins in the family (but not in EPCR) is usually used for antigen binding.

Like the CD1 series, EPCR has a lipid in the corresponding groove. The bound lipid in EPCR is usually phosphatidylcholine, but it may be phosphatidylethanolamine, and it contributes to protein C binding, though probably not through direct contact ^[4]

Clinical significance

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 Plasmodium falciparum erythrocyte membrane protein 1 (PfEMP1) family use EPCR of the host as a receptor.^[5]

References

↑ 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.
↑ 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.
↑ ^3.0 ^3.1 ^3.2 "Entrez Gene: PROCR protein C receptor, endothelial (EPCR)".
↑ Esmon CT (May 2004). "Structure and functions of the endothelial cell protein C receptor". Critical Care Medicine. 32 (5 Suppl): S298–301. doi:10.1097/01.CCM.0000126128.64614.81. PMID 15118534.
↑ Turner L, Lavstsen T, Berger SS, Wang CW, Petersen JE, Avril M, Brazier AJ, Freeth J, Jespersen JS, Nielsen MA, Magistrado P, Lusingu J, Smith JD, Higgins MK, Theander TG (2013). "Severe malaria is associated with parasite binding to endothelial protein C receptor". Nature. doi:10.1038/nature12216. PMC 3870021.

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.

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[pmid7929370-1] 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.

[pmid10518938-2] 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.

[entrez-3] 3.0 ^3.1 ^3.2 "Entrez Gene: PROCR protein C receptor, endothelial (EPCR)".

[4] Esmon CT (May 2004). "Structure and functions of the endothelial cell protein C receptor". Critical Care Medicine. 32 (5 Suppl): S298–301. doi:10.1097/01.CCM.0000126128.64614.81. PMID 15118534.

[5] Turner L, Lavstsen T, Berger SS, Wang CW, Petersen JE, Avril M, Brazier AJ, Freeth J, Jespersen JS, Nielsen MA, Magistrado P, Lusingu J, Smith JD, Higgins MK, Theander TG (2013). "Severe malaria is associated with parasite binding to endothelial protein C receptor". Nature. doi:10.1038/nature12216. PMC 3870021.

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v t e Proteins: clusters of differentiation (see also list of human clusters of differentiation)
1-50	CD1 a-c 1A 1D 1E CD2 CD3 γ δ ε CD4 CD5 CD6 CD7 CD8 a CD9 CD10 CD11 a b c d CD13 CD14 CD15 CD16 A B CD18 CD19 CD20 CD21 CD22 CD23 CD24 CD25 CD26 CD27 CD28 CD29 CD30 CD31 CD32 A B CD33 CD34 CD35 CD36 CD37 CD38 CD39 CD40 CD41 CD42 a b c d CD43 CD44 CD45 CD46 CD47 CD48 CD49 a b c d e f CD50
51-100	CD51 CD52 CD53 CD54 CD55 CD56 CD57 CD58 CD59 CD61 CD62 E L P CD63 CD64 A B C CD66 a b c d e f CD68 CD69 CD70 CD71 CD72 CD73 CD74 CD78 CD79 a b CD80 CD81 CD82 CD83 CD84 CD85 a d e h j k CD86 CD87 CD88 CD89 CD90 CD91 - CD92 CD93 CD94 CD95 CD96 CD97 CD98 CD99 CD100
101-150	CD101 CD102 CD103 CD104 CD105 CD106 CD107 a b CD108 CD109 CD110 CD111 CD112 CD113 CD114 CD115 CD116 CD117 CD118 CD119 CD120 a b CD121 a b CD122 CD123 CD124 CD125 CD126 CD127 CD129 CD130 CD131 CD132 CD133 CD134 CD135 CD136 CD137 CD138 CD140b CD141 CD142 CD143 CD144 CD146 CD147 CD148 CD150
151-200	CD151 CD152 CD153 CD154 CD155 CD156 a b c CD157 CD158 (a d e i k) CD159 a c CD160 CD161 CD162 CD163 CD164 CD166 CD167 a b CD168 CD169 CD170 CD171 CD172 a b g CD174 CD177 CD178 CD179 a b CD180 CD181 CD182 CD183 CD184 CD185 CD186 CD191 CD192 CD193 CD194 CD195 CD196 CD197 CDw198 CDw199 CD200
201-250	CD201 CD202b CD204 CD205 CD206 CD207 CD208 CD209 CDw210 a b CD212 CD213a 1 2 CD217 CD218 (a b) CD220 CD221 CD222 CD223 CD224 CD225 CD226 CD227 CD228 CD229 CD230 CD233 CD234 CD235 a b CD236 CD238 CD239 CD240CE CD240D CD241 CD243 CD244 CD246 CD247 - CD248 CD249
251-300	CD252 CD253 CD254 CD256 CD257 CD258 CD261 CD262 CD263 CD264 CD265 CD266 CD267 CD268 CD269 CD271 CD272 CD273 CD274 CD275 CD276 CD278 CD279 CD280 CD281 CD282 CD283 CD284 CD286 CD288 CD289 CD290 CD292 CDw293 CD294 CD295 CD297 CD298 CD299
301-350	CD300A CD301 CD302 CD303 CD304 CD305 CD306 CD307 CD309 CD312 CD314 CD315 CD316 CD317 CD318 CD320 CD321 CD322 CD324 CD325 CD326 CD328 CD329 CD331 CD332 CD333 CD334 CD335 CD336 CD337 CD338 CD339 CD340 CD344 CD349 CD350

Endothelial protein C receptor

Contents

Structure

Clinical significance

References

Further reading

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