Thrombomodulin functions as acofactorin thethrombin-induced activation ofprotein Cin theanticoagulantpathway by forming a 1:1 stoichiometric complex with thrombin. This raises the speed of protein C activation thousandfold. Thrombomodulin-bound thrombin has procoagulant effect at the same time by inhibiting fibrinolysis by cleavingthrombin-activatable fibrinolysis inhibitor(TAFI, aka carboxypeptidase B2) into its active form.[citation needed]
Thrombomodulin is aglycoproteinon the surface of endothelial cells that, in addition to binding thrombin, regulatesC3binactivation by factor I. Mutations in the thrombomodulin gene (THBD) have also been reported to be associated withatypical hemolytic-uremic syndrome(aHUS).[10]
Theantigendescribed as BDCA-3[11]has turned out to be identical to thrombomodulin.[12]Thus, it was revealed that this molecule also occurs on a very rare (0.02%) subset of humandendritic cellscalled MDC2. Its function on these cells is unknown.[citation needed]
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^Wen DZ, Dittman WA, Ye RD, Deaven LL, Majerus PW, Sadler JE (July 1987). "Human thrombomodulin: complete cDNA sequence and chromosome localization of the gene".Biochemistry.26(14): 4350–4357.doi:10.1021/bi00388a025.PMID2822087.
^Dzionek A, Inagaki Y, Okawa K, Nagafune J, Röck J, Sohma Y, et al. (December 2002). "Plasmacytoid dendritic cells: from specific surface markers to specific cellular functions".Human Immunology.63(12): 1133–1148.doi:10.1016/S0198-8859(02)00752-8.PMID12480257.
Ohlin AK, Norlund L, Marlar RA (July 1997). "Thrombomodulin gene variations and thromboembolic disease".Thrombosis and Haemostasis.78(1): 396–400.doi:10.1055/s-0038-1657559.PMID9198186.S2CID25122238.
Van de Wouwer M, Collen D, Conway EM (August 2004). "Thrombomodulin-protein C-EPCR system: integrated to regulate coagulation and inflammation".Arteriosclerosis, Thrombosis, and Vascular Biology.24(8): 1374–1383.doi:10.1161/01.ATV.0000134298.25489.92.PMID15178554.
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Wen DZ, Dittman WA, Ye RD, Deaven LL, Majerus PW, Sadler JE (July 1987). "Human thrombomodulin: complete cDNA sequence and chromosome localization of the gene".Biochemistry.26(14): 4350–4357.doi:10.1021/bi00388a025.PMID2822087.
Shirai T, Shiojiri S, Ito H, Yamamoto S, Kusumoto H, Deyashiki Y, et al. (February 1988). "Gene structure of human thrombomodulin, a cofactor for thrombin-catalyzed activation of protein C".Journal of Biochemistry.103(2): 281–285.CiteSeerX10.1.1.1006.6759.doi:10.1093/oxfordjournals.jbchem.a122261.PMID2836377.
Yonezawa S, Maruyama I, Tanaka S, Nakamura T, Sato E (August 1988). "Immunohistochemical localization of thrombomodulin in chorionic diseases of the uterus and choriocarcinoma of the stomach. A comparative study with the distribution of human chorionic gonadotropin".Cancer.62(3): 569–576.doi:10.1002/1097-0142(19880801)62:3<569::AID-CNCR2820620322>3.0.CO;2-T.PMID2839283.S2CID28744944.
Srinivasan J, Hu S, Hrabal R, Zhu Y, Komives EA, Ni F (November 1994). "Thrombin-bound structure of an EGF subdomain from human thrombomodulin determined by transferred nuclear Overhauser effects".Biochemistry.33(46): 13553–13560.doi:10.1021/bi00250a007.PMID7947766.
Yasuda K, Espinosa R, Davis EM, Le Beau MM, Bell GI (September 1993). "Human somatostatin receptor genes: localization of SSTR5 to human chromosome 20p11.2".Genomics.17(3): 785–786.doi:10.1006/geno.1993.1410.PMID8244401.
Yamamoto S, Mizoguchi T, Tamaki T, Ohkuchi M, Kimura S, Aoki N (April 1993). "Urinary thrombomodulin, its isolation and characterization".Journal of Biochemistry.113(4): 433–440.doi:10.1093/oxfordjournals.jbchem.a124063.PMID8390446.