• Anderson, Jr F.A., Wheeler, H.B., Goldberg, R.J., Hosmer, D.W., Patwardhan, N.A., Jovanovic, B., Forcier, A. & Dalen, J.E. (1991) A population-based perspective of the hospital incidence and case-fatality rates of deep vein thrombosis and pulmonary embolism. The Worcester DVT Study. Archives of Internal Medicine, 151, 933938.
  • Bertina, R.M., Ploos van Amstel, H.K., van Wijngaarden, A., Coenen, J., Leemhuis, M.P., Deutz-Terlouw, P.P., van der Linden, I.K. & Reitsma, P.H. (1990) Heerlen polymorphism of protein S, an immunologic polymorphism due to dimorphism of residue 460. Blood, 76, 538548.
  • Borgel, D., Duchemin, J., Alhenc-Gelas, M., Matheron, C., Aiach, M. & Gandrille, S. (1996) Molecular basis for protein S hereditary deficiency: genetic defects observed in 118 patients with type I and type IIa deficiencies. The French Network on Molecular Abnormalities Responsible for Protein C and Protein S Deficiencies. The Journal of Laboratory and Clinical Medicine, 128, 218227.
  • Comp, P.C., Thurnau, G.R., Welsh, J. & Esmon, C.T. (1986) Functional and immunologic protein S levels are decreased during pregnancy. Blood, 68, 881885.
  • Dahlback, B. & Stenflo, J. (1981) High molecular weight complex in human plasma between vitamin K-dependent protein S and complement component C4b-binding protein. Proceedings of the National Academy of Sciences of the United States of America, 78, 25122516.
  • De Stefano, V., Finazzi, G. & Mannucci, P.M. (1996) Inherited thrombophilia: pathogenesis, clinical syndromes, and management. Blood, 87, 35313544.
  • Duchemin, J., Gandrille, S., Borgel, D., Feurgard, P., Alhenc-Gelas, M., Matheron, C., Dreyfus, M., Dupuy, E., Juhan-Vague, I. & Aiach, M. (1995) The Ser 460 to Pro substitution of the protein S alpha (PROS1) gene is a frequent mutation associated with free protein S (type IIa) deficiency. Blood, 86, 34363443.
  • Dykes, A.C., Walker, I.D., McMahon, A.D., Islam, S.I. & Tait, R.C. (2001) A study of Protein S antigen levels in 3788 healthy volunteers: influence of age, sex and hormone use, and estimate for prevalence and deficiency state. British Journal of Haematology, 113, 636641.
  • Espinosa-Parrilla, Y., Morell, M., Souto, J.C., Tirado, I., Fontcuberta, J., Estivill, X. & Sala, N. (1999) Protein S gene analysis reveals the presence of a cosegregating mutation in most pedigrees with type I but not type III PS deficiency. Human Mutation, 14, 3039.
  • Faioni, E.M., Valsecchi, C., Palla, A., Taioli, E., Razzari, C. & Mannucci, P.M. (1997) Free protein S deficiency is a risk factor for venous thrombosis. Thrombosis and Haemostasis, 78, 13431346.
  • Gladson, C.L., Scharrer, I., Hach, V., Beck, K.H. & Griffin, J.H. (1988) The frequency of type I heterozygous protein S and protein C deficiency in 141 unrelated young patients with venous thrombosis. Thrombosis and Haemostasis, 59, 1822.
  • Heijboer, H., Brandjes, D.P., Buller, H.R., Sturk, A. & Ten Cate, J.W. (1990) Deficiencies of coagulation-inhibiting and fibrinolytic proteins in outpatients with deep-vein thrombosis. New England Journal of Medicine, 323, 15121516.
  • Henkens, C.M., Bom, V.J., Van der Schaaf, W., Pelsma, P.M., Sibinga, C.T., de Kam, P.J. & Van der Meer, J. (1995) Plasma levels of protein S, protein C, and factor X: effects of sex, hormonal state and age. Thrombosis and Haemostasis, 74, 12711275.
  • Koster, T., Rosendaal, F.R., Briet, E., van der Meer, F.J., Colly, L.P., Trienekens, P.H., Poort, S.R., Reitsma, P.H. & Vandenbroucke, J.P. (1995) Protein C deficiency in a controlled series of unselected outpatients: an infrequent but clear risk factor for venous thrombosis(Leiden Thrombophilia Study). Blood, 85, 27562761.
  • Makris, M., Leach, M., Beauchamp, N.J., Daly, M.E., Cooper, P.C., Hampton, K.K., Bayliss, P., Peake, I.R., Miller, G.J. & Preston, F.E. (2000) Genetic analysis, phenotypic diagnosis, and risk of venous thrombosis in families with inherited deficiencies of protein S. Blood, 95, 19351941.
  • Martinelli, I., Mannucci, P.M., De Stefano, V., Taioli, E., Rossi, V., Crosti, F., Paciaroni, K., Leone, G. & Faioni, E.M. (1998) Different risks of thrombosis in four coagulation defects associated with inherited thrombophilia: a study of 150 families. Blood, 92, 23532358.
  • Mateo, J., Oliver, A., Borrell, M., Sala, N. & Fontcuberta, J. (1998) Increased risk of venous thrombosis in carriers of natural anticoagulant deficiencies. Results of the family studies of the Spanish Multicenter Study on Thrombophilia (EMET study). Blood Coagulation and Fibrinolysis, 9, 7178.
  • Mayer, S.A., Sacco, R.L., Hurlet-Jensen, A., Shi, T. & Mohr, J.P. (1993) Free protein S deficiency in acute ischemic stroke. A case-control study. Stroke, 24, 224227.
  • Nordstrom, M., Lindblad, B., Bergqvist, D. & Kjellstrom, T. (1992) A prospective study of the incidence of deep-vein thrombosis within a defined urban population. Journal of Internal Medicine, 232, 155160.
  • Pabinger, I., Brucker, S., Kyrle, P.A., Schneider, B., Korninger, H.C., Niessner, H. & Lechner, K. (1992) Hereditary deficiency of antithrombin III, protein C and protein S: prevalence in patients with a history of venous thrombosis and criteria for rational patient screening. Blood Coagulation and Fibrinolysis, 3, 547553.
  • Rudnicka, A.R., Miller, G.J., Nelson, T., Doray, D. & Comp, P.C. (2001) An association between plasma free protein S concentration and risk of coronary heart disease in middle-aged men. Thrombosis Research, 101, 111.
  • Sacco, R.L., Owen, J., Mohr, J.P., Tatemichi, T.K. & Grossman, B.A. (1989) Free protein S deficiency: a possible association with cerebrovascular occlusion. Stroke, 20, 16571661.
  • Schattner, A., Kasher, I. & Berrebi, A. (1997) Causes and outcome of deep-vein thrombosis in otherwise-healthy patients under 50 years. QJM: An International Journal of Medicine, 90, 283287.
  • Simmonds, R.E., Zoller, B., Ireland, H., Thompson, E., de Frutos, P.G., Dahlback, B. & Lane, D.A. (1997) Genetic and phenotypic analysis of a large (122-member) protein S-deficient kindred provides an explanation for the familial coexistence of type I and type III plasma phenotypes. Blood, 89, 43644370.
  • Simmonds, R.E., Ireland, H., Lane, D.A., Zoller, B., Garcia de Frutos, P. & Dahlback, B. (1998) Clarification of the risk for venous thrombosis associated with hereditary protein S deficiency by investigation of a large kindred with a characterized gene defect. Annals of Internal Medicine, 128, 814.
  • Zoller, B., Garcia de Frutos, P. & Dahlback, B. (1995) Evaluation of the relationship between protein S and C4b-binding protein isoforms in hereditary protein S deficiency demonstrating type I and type III deficiencies to be phenotypic variants of the same genetic disease. Blood, 85, 35243531.