Endothelial protein C receptor (EPCR) also known as activated protein C receptor (APC receptor) is a protein that in humans is encoded by the PROCRgene.[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.
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]
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↑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.
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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. PMID10570964.
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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. PMID11099506.
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