Ишемический инсульт в молодом возрасте и тромбофилии, обусловленные дефицитом или аномалиями физиологических антикоагулянтов

1. Franchini M, Veneri D. Inherited thrombophilia: an update. Clin Lab 2005; 51: 357–65.
2. Nachman RL, Silverstein R. Hypercoagulable states. Ann Intern Med 1993; 119: 819–7.
3. Schafer AI. The hypercoagulable states. Ann Intern Med 1985; 102: 814–28.
4. Jordan FLJ, Nandorff A. The familial tendency in thromboembolic disease. Acta Med Scand 1956; 156: 267–75.
5. Egeberg O. Inherited antithrombin deficiency causing thrombophilia. Thromb Diath Haemorgh 1965; 13: 516–30.
6. Egeberg O. Proceedings: Inherited antithrombin III deficiency and thromboembolism. Thromb Diath Haemorgh 1975; 34: 366.
7. Manucci PM. The molecular basis of inherited thrombophilia. Vox Sang 2000; 78: 39–5.
8. Сушкевич Г.Н. Тромбогенерирующие системы при тромбофилиях различного генеза. Лаб. мед. 2009; 10: 11–22. / Sushkevich G.N. Trombogeneriruiushchie sistemy pri trombofiliiakh razlichnogo geneza. Lab. med. 2009; 10: 11–22. [in Russian]
9. Патрушев Л.И. Тромбофилические состояния и современные методы их диагностики. РМЖ. 1998; 6 (3): 181–5. / Patrushev L.I. Trombofilicheskie sostoianiia i sovremennye metody ikh diagnostiki. RMZh. 1998; 6 (3): 181–5. [in Russian]
10. Васильев С.А., Виноградов В.Л., Гемджян Э.Г. и др. Сочетанные генетические и приобретенные формы тромбофилий. 4-я Всерос. конф. «Клиническая гемостазиология в сердечно-сосудистой хирургии» (с международным участием). М.: НЦССХ им. А.Н.Бакулева, 2009; с. 84–5. / Vasil'ev S.A., Vinogradov V.L., Gemdzhian E.G. i dr. Sochetannye geneticheskie i priobretennye formy trombofilii. 4-ia Vseros. konf. «Klinicheskaia gemostaziologiia v serdechno-sosudistoi khirurgii» (s mezhdunarodnym uchastiem). M.: NTsSSKh im. A.N.Bakuleva, 2009; s. 84–5. [in Russian]
11. Barinagarrementeria F, Cantu-Brito C, De La Pena A, Izaguirre R. Prothrombotic states in young people with idiopathic stroke. A prospective study. Stroke 1994; 25 (2): 287–90.
12. Bock S, Harris J, Balazs I, Trent J. Assignment of the human antithrombin III structural gene to chromosome 1q23-25. Cytogenet Cell Genet 1985; 39: 67–9.
13. Kobelt K, Biasiutti FD, Mattle HP et al. Protein Z in ischaemic stroke. Br J Haematol 2001; 114: 169–73.
14. Spek CA, Reitsma PH. Genetic risk factors for venous thrombosis. Mol Genet Metab 2000; 71: 51–61.
15. Lane DA, Olds RJ, Boisclair M et al. Antithrombin III mutation database: first update. For the Thrombin and its Inhibitors Subcommittee of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1993; 70 (2): 361–9.
16. Lane D, Bayston T, Olds R et al. Antithrombin mutation database: 2nd (1997) update. For the Plasma Coagulation Inhibitors Subcommittee of the Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1997; 77: 197–211.
17. Лагутина Н.Я., Федулова Г.А. Антитромбин III. Обзор. Пробл. гематологии. 1982; 3: 42–50. / Lagutina N.Ia., Fedulova G.A. Antitrombin III. Obzor. Probl. gematologii. 1982; 3: 42–50. [in Russian]
18. Thaler E, Lechner K. Antithrombin III deficiency and thromboembolism. Clin Haematol 1981; 10 (2): 369–90.
19. Martinelli I, Mannucci PM, De Stefano V et al. Different risks of thrombosis in four coagulation defects associated with inherited thrombophilia: a study of 150 families. Blood 1998; 92 (7): 2353–8.
20. Simioni P, Sanson BJ, Prandoni P et al. Incidence of venous thromboembolism in families with inherited thrombophilia. Thromb Haemost 1999; 81 (2): 198–202.
21. Tait RC, Walker ID, Perry DJ et al. Prevalence of antithrombin deficiency in the healthy population. Br J Haematol 1994; 87 (1): 106–12.
22. Moster M. Coagulopathies and arterial stroke. J Neuro-Ophthalmol 2003; 23 (1): 63–71.
23. Tollefsen DM, Majerus DW, Blank MK. Heparin cofactor II. Purification and properties of a heparin-dependent inhibitor of thrombin in human plasma. J Biol Chem 1982; 257 (5): 2162–9.
24. Tollefsen DM. Heparin cofactor II modulates the response to vascular injury. Arterioscler Thromb Vasc Biol 2007; 27: 454–60.
25. Tollefsen DM. Heparin cofactor II deficiency. Arch Pathol Lab Med 2002; 126: 1394–400.
26. Schwarz HP, Fischer M, Hopmeier P et al. Plasma protein S deficiency in familial thrombotic disease. Blood 1984; 64 (6): 1297–300.
27. Tran TH, Duckert F. Influence of heparin cofactor II (HCII) on the determination of antithrombin III (AT). Thromb Res. 1985; 40 (4): 571–6.
28. Huntington JA, Kjellberg M, Stenflo J. Crystal structure of protein C inhibitor provides insights into hormone binding and heparin activation. Structure 2003; 11: 205–15.
29. Dhainaut J-F, Yan В, Cariou A, Mira J-P. Soluble thrombomodulin, plasma-derived unactivated protein C, and recombinant human activated protein С in sepsis. Crit Care Med 2002; 30: 318–24.
30. Esmon C. The protein С pathway. Crit Care Med 2000; 28: 44–8.
31. Pike RN, Buckle AM, le Bonniec BF, Church FC. Control of the coagulation system by serpins. Getting by with a little help from glycosaminoglycans. FEBS J 2005; 272: 4842–51.
32. Aznar J, Espana F, Estelles A, Royo M. Heparin stimulation of the inhibition of activated protein C and other enzymes by human protein C inhibitor – influence of the molecular weight of heparin and ionic strength. Thromb Haemost 1996; 76: 983–8.
33. Dahlback B, Carlsson M, Svensson PJ. Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: prediction of a cofactor to activated protein C. Proc Natl Acad Sci USA 1993; 90 (3): 1004–8.
34. Bertina RM, Broekmans AW, van der Linden IK, Mertens K. Protein C deficiency in a Dutch family with thrombotic disease. Thromb Haemost 1982; 48 (1): 1–5.
35. Bertina RM, Broekmans AW, Krommenhoek-van Es C, van Wijngaarden A. The use of a functional and immunologic assay for plasma protein C in the study of the heterogeneity of congenital protein C deficiency. Thromb Haemost 1984; 51 (1): 1–5.
36. Comp PC, Nixon RR, Esmon CT. Determination of functional levels of protein C, an antithrombotic protein, using thrombin-thrombomodulin complex. Blood 1984; 63 (1): 15–21.
37. Comp PC, Esman CT. Recurrent venous thromboembolism in patients with a partial deficiency of protein C. N Engl J Med 1984; 311 (24): 1525–28.
38. Patracchini P, Aiello V, Palazzi P et al. Sublocalization of the human protein C gene on chromosome 2q13-q14. Hum Genet 1989; 81 (2): 191–2.
39. De Stefano V, Rossi E, Paciaroni K, Leone G. Screening for inherited thrombophilia: indications and therapeutic implications. Haematologica 2002; 87: 1095–108.
40. Griffin JH, Evatt B, Zimmermann TS et al. Deficiency of protein C in congenital thrombotic disease. J Clin Invest 1981; 68: 1370–73.
41. Pabinger J, Kurie PA, Heistinger M et al. The risc of thromboembolism in asymptomatic patients with protein C and protein S debiciencg. Thromb Haemost 1994; 71: 441–5.
42. Miletich JP, Sherman L, Broze GJJ. Absence of thrombosis in subjects with heterozygous protein C deficiency. N Engl J Med 1987; 317: 991–6.
43. Griffin JH. Clinical studies of protein C. Semin Thromb Hemost 1984; 10 (2): 162–6.
44. Tait RC, Walker ID, Reitsma PH et al. Prevalence of protein C deficiency in the healthy population. Thromb Haemost 1995; 73 (1): 87–93.
45. Meijer-Huizinga F, Mertens K, van Mourik JA. Isolation and characterization of single-chain protein S. Thromb Haemost 1994; 72 (3): 408–14.
46. Walker FJ. Regulation of activated protein C by a new protein. A possible function for bovine protein S. J Biol Chem 1980; 255 (12): 5521–4.
47. Walker FJ. Regulation of activated protein C by protein S. The role of phospholipid in factor Va inactivation. J Biol Chem 1981; 256 (21): 11128–31.
48. Walker FJ, Chavin SI, Fay PJ. Inactivation of factor VIII by activated protein C and protein S. Arch Biochem Biophys 1987; 252 (1): 322–8.
49. Schwarz HP, Fischer M, Hopmeier P et al. Plasma protein S deficiency in familial thrombotic disease. Blood 1984; 64 (6): 1297–300.
50. Allaart CF, Aronson DC, Ruys T et al. Hereditary protein S deficiency in young adults with arterial occlusive disease. Thromb Haemost 1990; 64 (2): 206–10.
51. Broekmans AW, Bertina RM, Reinalda-Poot J et al. Hereditary protein S deficiency and venous thrombo-embolism. A study in three Dutch families. Thromb Haemost 1985; 53 (2): 273–7.
52. Makris M, Leach M, Beauchamp NJ et al. Genetic analysis, phenotypic diagnosis, and risk of venous thrombosis in families with inherited deficiencies of protein S. Blood 2000; 95 (6): 1935–41.
53. Long GL, Marshall A, Gardner JC, Naylor SL. Genes for human vitamin K-dependent plasma proteins C and S are located on chromosomes 2 and 3, respectively. Somat Cell Mol Genet 1988; 14 (1): 93–8.
54. Watkins PC, Eddy R, Beck AK et al. DNA sequence and regional assignment of the human follicle-stimulating hormone beta-subunit gene to the short arm of human chromosome 11. DNA 1987; 6 (3): 205–12.
55. Watkins PC, Eddy R, Fukushima Y et al. The gene for protein S maps near the centromere of human chromosome 3. Blood 1988; 71 (1): 238–41.
56. Engesser L, Broekmans AW, Briet E et al. Hereditary protein S deficiency: clinical manifestations. Ann Intern Med 1987; 106 (5): 677–82.
57. Girolami A, Simioni P, Lazzaro AR, Cordiano I. Severe arterial cerebral thrombosis in a patient with protein S deficiency (moderately reduced total and markedly reduced free protein S): a family study. Thromb Haemost 1989; 61 (1): 144–7.
58. Sie P, Boneu B, Bierme R et al. Arterial thrombosis and protein S deficiency. Thromb Haemost 1989; 62 (3): 1040.
59. Wiesel ML, Borg JY, Grunebaum L et al. Influence of protein S deficiency on the arterial thrombosis risk. Presse Med 1991; 20 (22): 1023–7.
60. Douay X, Lucas C, Caron C et al. Antithrombin, protein C and protein S levels in 127 consecutive young adults with ischemic stroke. Acta Neurol Scand 1998; 98 (2): 124–7.
61. Mayer SA, Sacco RL, Hurlet-Jensen A et al. Free protein S deficiency in acute ischemic stroke. A case-control study. Stroke 1993; 24 (2): 224–7.
62. Broze GJ, Miletich J.P. Human protein Z. J Clin Invest 1984; 73: 933–38.
63. Hogg PJ, Stenflo J. Interaction of vitamin K-dependent protein Z with thrombin. J Biol Chem 1990; 266: 1053–58.
64. Hogg PJ, Stenflo J. Interaction of human protein Z with thrombin: evaluation of the species difference in the interaction between bovine and human protein Z and thrombin. Biochem Biophys Res Commun 1991; 178: 801–7.
65. Kempkes-Matthes B, Walmrath D, Matthes KJ. Protein Z deficiency: a new cause of bleeding tendency. Thromb Res 1995; 79: 49–55.
66. Broze GJJr. Protein Z dependent regulation of coagulation. Thromb Haemost 2001; 86: 8–13.
67. Kemkes-Matthes B, Souri M, Ichinose A, Matthes KJ. First cases of homozygous combined protein Z- and factor V Leiden-mutation in humans. Blood 2000; 96: 74b. Abstract.
68. Kemkes-Matthes B, Nees M, Kuhnel G et al. Protein Z influences prothrombotic phenotype of factor V Leiden in humans. Thromb Res 2002; 106: 183–85.
69. McQuillan AM, Eikelboom JW, Hankey GJ et al. Protein Z in ischemic stroke and its etiologic subtypes. Stroke 2003; 34: 2415–19.
70. Fujimaki K, Yamazaki T, Taniwaki M, Ichinose A. The gene for human protein Z is localized to chromosome 13 at band q34 and is coded by eight regular exons and one alternative exon. Biochemistry 1998; 37: 6838–46.
71. Vasse M, Guegan-Massardier E, Borg JY et al. Frequency of protein Z deficiency in patients with ischaemic stroke. Lancet 2001; 357: 933–34.
72. Heeb MJ, Paganini-Hill A, Griffin JH, Fischer M. Low protein Z levels and risk of ischaemic stroke: differences by diabetic status and gender. Blood Cells Mol Dis 2002; 29: 139–44.
73. Lopaciuk S, Bykowska K, Kwiecinski H et al. Protein Z in young survivors of ischaemic stroke. Thromb Haemost 2002; 88: 536.
74. Личи К, Кропп С, Донг-Си Т и др. Уровень протеина Z в плазме и риск развития ишемии головного мозга у молодых лиц зависят от варианта полиморфизма гена, кодирующего этот белок. Stroke. Российское издание. 2004; 5: 64–70. / Lichi K, Kropp S, Dong-Si T i dr. Uroven' proteina Z v plazme i risk razvitiia ishemii golovnogo mozga u molodykh lits zavisiat ot varianta polimorfizma gena, kodiruiushchego etot belok. Stroke. Rossiiskoe izdanie. 2004; 5: 64–70. [in Russian]
75. Bird P. Thrombomodulin. Haematol Rev 1996; 9: 251–74.
76. Кудряшева О.В., Затейщиков Д.А., Сидоренко Б.А. Эндотелиальный гемостаз: система тромбомодулина и ее роль в развитии атеросклероза и его осложнений. Кардиология. 2000; 40 (8): 65–70. / Kudriasheva O.V., Zateishchikov D.A., Sidorenko B.A. Endotelial'nyi gemostaz: sistema trombomodulina i ee rol' v razvitii ateroskleroza i ego oslozhnenii. Kardiologiia. 2000; 40 (8): 65–70. [in Russian]
77. Bushnell CD, Goldstein LB. Diagnostic testing for coagulopathies in patients with ischemic stroke. Stroke 2000; 31: 3067–78.
78. Carod-Artal FJ, Nunes SV, Portugal D et al. Ischemic stroke subtypes and thrombophilia in young and elderly Brazilian stroke patients admitted to a rehabilitation hospital. Stroke 2005; 36 (9): 2012–4.
79. Ranzan J, Rotta NT. Ischemic stroke in children: a study of the associated alterations. Arq Neuropsiquiatr 2004; 62 (3A): 618–25.
80. Hellgren M, Svensson PJ, Dahlback B. Resistance to activated protein C as a basis for venous thromboembolism associated with pregnancy and contraceptives. Am J Obstet Gynecol 1995; 173: 210–13.
81. Gandrille S, Greengard JS, Alhenc-Gelas M et al. Incidence of activated protein C resistance caused by the ARG 506 GLN mutation in factor V in 113 unrelated symptomatic protein C-deficient patients. The French Network on the behalf of INSERM. Blood 1995; 86: 219–24.
82. Zoller B, Berntsdotter A, Garcia de Frutos P, Dahlback B. Resistance to activated protein C as an additional genetic risk factor in hereditary deficiency of protein S. Blood 1995; 85: 3518–23.
83. Alhenc-Gelas M, Aiach M, de Moerloose P. Venous thromboembolic disease: risk factors and laboratory investigation. Semin Vasc Med 2001; 1 (1): 81–8.
84. Varnai K, Gutler F, Szekely E. Hemorheological parameters in patients with thrombophilia. Biorheology 1999; 1–2: 148.

________________________________________________

1. Franchini M, Veneri D. Inherited thrombophilia: an update. Clin Lab 2005; 51: 357–65.
2. Nachman RL, Silverstein R. Hypercoagulable states. Ann Intern Med 1993; 119: 819–7.
3. Schafer AI. The hypercoagulable states. Ann Intern Med 1985; 102: 814–28.
4. Jordan FLJ, Nandorff A. The familial tendency in thromboembolic disease. Acta Med Scand 1956; 156: 267–75.
5. Egeberg O. Inherited antithrombin deficiency causing thrombophilia. Thromb Diath Haemorgh 1965; 13: 516–30.
6. Egeberg O. Proceedings: Inherited antithrombin III deficiency and thromboembolism. Thromb Diath Haemorgh 1975; 34: 366.
7. Manucci PM. The molecular basis of inherited thrombophilia. Vox Sang 2000; 78: 39–5.
8. Sushkevich G.N. Trombogeneriruiushchie sistemy pri trombofiliiakh razlichnogo geneza. Lab. med. 2009; 10: 11–22. [in Russian]
9. Patrushev L.I. Trombofilicheskie sostoianiia i sovremennye metody ikh diagnostiki. RMZh. 1998; 6 (3): 181–5. [in Russian]
10. Vasil'ev S.A., Vinogradov V.L., Gemdzhian E.G. i dr. Sochetannye geneticheskie i priobretennye formy trombofilii. 4-ia Vseros. konf. «Klinicheskaia gemostaziologiia v serdechno-sosudistoi khirurgii» (s mezhdunarodnym uchastiem). M.: NTsSSKh im. A.N.Bakuleva, 2009; s. 84–5. [in Russian]
11. Barinagarrementeria F, Cantu-Brito C, De La Pena A, Izaguirre R. Prothrombotic states in young people with idiopathic stroke. A prospective study. Stroke 1994; 25 (2): 287–90.
12. Bock S, Harris J, Balazs I, Trent J. Assignment of the human antithrombin III structural gene to chromosome 1q23-25. Cytogenet Cell Genet 1985; 39: 67–9.
13. Kobelt K, Biasiutti FD, Mattle HP et al. Protein Z in ischaemic stroke. Br J Haematol 2001; 114: 169–73.
14. Spek CA, Reitsma PH. Genetic risk factors for venous thrombosis. Mol Genet Metab 2000; 71: 51–61.
15. Lane DA, Olds RJ, Boisclair M et al. Antithrombin III mutation database: first update. For the Thrombin and its Inhibitors Subcommittee of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1993; 70 (2): 361–9.
16. Lane D, Bayston T, Olds R et al. Antithrombin mutation database: 2nd (1997) update. For the Plasma Coagulation Inhibitors Subcommittee of the Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1997; 77: 197–211.
17. Lagutina N.Ia., Fedulova G.A. Antitrombin III. Obzor. Probl. gematologii. 1982; 3: 42–50. [in Russian]
18. Thaler E, Lechner K. Antithrombin III deficiency and thromboembolism. Clin Haematol 1981; 10 (2): 369–90.
19. Martinelli I, Mannucci PM, De Stefano V et al. Different risks of thrombosis in four coagulation defects associated with inherited thrombophilia: a study of 150 families. Blood 1998; 92 (7): 2353–8.
20. Simioni P, Sanson BJ, Prandoni P et al. Incidence of venous thromboembolism in families with inherited thrombophilia. Thromb Haemost 1999; 81 (2): 198–202.
21. Tait RC, Walker ID, Perry DJ et al. Prevalence of antithrombin deficiency in the healthy population. Br J Haematol 1994; 87 (1): 106–12.
22. Moster M. Coagulopathies and arterial stroke. J Neuro-Ophthalmol 2003; 23 (1): 63–71.
23. Tollefsen DM, Majerus DW, Blank MK. Heparin cofactor II. Purification and properties of a heparin-dependent inhibitor of thrombin in human plasma. J Biol Chem 1982; 257 (5): 2162–9.
24. Tollefsen DM. Heparin cofactor II modulates the response to vascular injury. Arterioscler Thromb Vasc Biol 2007; 27: 454–60.
25. Tollefsen DM. Heparin cofactor II deficiency. Arch Pathol Lab Med 2002; 126: 1394–400.
26. Schwarz HP, Fischer M, Hopmeier P et al. Plasma protein S deficiency in familial thrombotic disease. Blood 1984; 64 (6): 1297–300.
27. Tran TH, Duckert F. Influence of heparin cofactor II (HCII) on the determination of antithrombin III (AT). Thromb Res. 1985; 40 (4): 571–6.
28. Huntington JA, Kjellberg M, Stenflo J. Crystal structure of protein C inhibitor provides insights into hormone binding and heparin activation. Structure 2003; 11: 205–15.
29. Dhainaut J-F, Yan В, Cariou A, Mira J-P. Soluble thrombomodulin, plasma-derived unactivated protein C, and recombinant human activated protein С in sepsis. Crit Care Med 2002; 30: 318–24.
30. Esmon C. The protein С pathway. Crit Care Med 2000; 28: 44–8.
31. Pike RN, Buckle AM, le Bonniec BF, Church FC. Control of the coagulation system by serpins. Getting by with a little help from glycosaminoglycans. FEBS J 2005; 272: 4842–51.
32. Aznar J, Espana F, Estelles A, Royo M. Heparin stimulation of the inhibition of activated protein C and other enzymes by human protein C inhibitor – influence of the molecular weight of heparin and ionic strength. Thromb Haemost 1996; 76: 983–8.
33. Dahlback B, Carlsson M, Svensson PJ. Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: prediction of a cofactor to activated protein C. Proc Natl Acad Sci USA 1993; 90 (3): 1004–8.
34. Bertina RM, Broekmans AW, van der Linden IK, Mertens K. Protein C deficiency in a Dutch family with thrombotic disease. Thromb Haemost 1982; 48 (1): 1–5.
35. Bertina RM, Broekmans AW, Krommenhoek-van Es C, van Wijngaarden A. The use of a functional and immunologic assay for plasma protein C in the study of the heterogeneity of congenital protein C deficiency. Thromb Haemost 1984; 51 (1): 1–5.
36. Comp PC, Nixon RR, Esmon CT. Determination of functional levels of protein C, an antithrombotic protein, using thrombin-thrombomodulin complex. Blood 1984; 63 (1): 15–21.
37. Comp PC, Esman CT. Recurrent venous thromboembolism in patients with a partial deficiency of protein C. N Engl J Med 1984; 311 (24): 1525–28.
38. Patracchini P, Aiello V, Palazzi P et al. Sublocalization of the human protein C gene on chromosome 2q13-q14. Hum Genet 1989; 81 (2): 191–2.
39. De Stefano V, Rossi E, Paciaroni K, Leone G. Screening for inherited thrombophilia: indications and therapeutic implications. Haematologica 2002; 87: 1095–108.
40. Griffin JH, Evatt B, Zimmermann TS et al. Deficiency of protein C in congenital thrombotic disease. J Clin Invest 1981; 68: 1370–73.
41. Pabinger J, Kurie PA, Heistinger M et al. The risc of thromboembolism in asymptomatic patients with protein C and protein S debiciencg. Thromb Haemost 1994; 71: 441–5.
42. Miletich JP, Sherman L, Broze GJJ. Absence of thrombosis in subjects with heterozygous protein C deficiency. N Engl J Med 1987; 317: 991–6.
43. Griffin JH. Clinical studies of protein C. Semin Thromb Hemost 1984; 10 (2): 162–6.
44. Tait RC, Walker ID, Reitsma PH et al. Prevalence of protein C deficiency in the healthy population. Thromb Haemost 1995; 73 (1): 87–93.
45. Meijer-Huizinga F, Mertens K, van Mourik JA. Isolation and characterization of single-chain protein S. Thromb Haemost 1994; 72 (3): 408–14.
46. Walker FJ. Regulation of activated protein C by a new protein. A possible function for bovine protein S. J Biol Chem 1980; 255 (12): 5521–4.
47. Walker FJ. Regulation of activated protein C by protein S. The role of phospholipid in factor Va inactivation. J Biol Chem 1981; 256 (21): 11128–31.
48. Walker FJ, Chavin SI, Fay PJ. Inactivation of factor VIII by activated protein C and protein S. Arch Biochem Biophys 1987; 252 (1): 322–8.
49. Schwarz HP, Fischer M, Hopmeier P et al. Plasma protein S deficiency in familial thrombotic disease. Blood 1984; 64 (6): 1297–300.
50. Allaart CF, Aronson DC, Ruys T et al. Hereditary protein S deficiency in young adults with arterial occlusive disease. Thromb Haemost 1990; 64 (2): 206–10.
51. Broekmans AW, Bertina RM, Reinalda-Poot J et al. Hereditary protein S deficiency and venous thrombo-embolism. A study in three Dutch families. Thromb Haemost 1985; 53 (2): 273–7.
52. Makris M, Leach M, Beauchamp NJ et al. Genetic analysis, phenotypic diagnosis, and risk of venous thrombosis in families with inherited deficiencies of protein S. Blood 2000; 95 (6): 1935–41.
53. Long GL, Marshall A, Gardner JC, Naylor SL. Genes for human vitamin K-dependent plasma proteins C and S are located on chromosomes 2 and 3, respectively. Somat Cell Mol Genet 1988; 14 (1): 93–8.
54. Watkins PC, Eddy R, Beck AK et al. DNA sequence and regional assignment of the human follicle-stimulating hormone beta-subunit gene to the short arm of human chromosome 11. DNA 1987; 6 (3): 205–12.
55. Watkins PC, Eddy R, Fukushima Y et al. The gene for protein S maps near the centromere of human chromosome 3. Blood 1988; 71 (1): 238–41.
56. Engesser L, Broekmans AW, Briet E et al. Hereditary protein S deficiency: clinical manifestations. Ann Intern Med 1987; 106 (5): 677–82.
57. Girolami A, Simioni P, Lazzaro AR, Cordiano I. Severe arterial cerebral thrombosis in a patient with protein S deficiency (moderately reduced total and markedly reduced free protein S): a family study. Thromb Haemost 1989; 61 (1): 144–7.
58. Sie P, Boneu B, Bierme R et al. Arterial thrombosis and protein S deficiency. Thromb Haemost 1989; 62 (3): 1040.
59. Wiesel ML, Borg JY, Grunebaum L et al. Influence of protein S deficiency on the arterial thrombosis risk. Presse Med 1991; 20 (22): 1023–7.
60. Douay X, Lucas C, Caron C et al. Antithrombin, protein C and protein S levels in 127 consecutive young adults with ischemic stroke. Acta Neurol Scand 1998; 98 (2): 124–7.
61. Mayer SA, Sacco RL, Hurlet-Jensen A et al. Free protein S deficiency in acute ischemic stroke. A case-control study. Stroke 1993; 24 (2): 224–7.
62. Broze GJ, Miletich J.P. Human protein Z. J Clin Invest 1984; 73: 933–38.
63. Hogg PJ, Stenflo J. Interaction of vitamin K-dependent protein Z with thrombin. J Biol Chem 1990; 266: 1053–58.
64. Hogg PJ, Stenflo J. Interaction of human protein Z with thrombin: evaluation of the species difference in the interaction between bovine and human protein Z and thrombin. Biochem Biophys Res Commun 1991; 178: 801–7.
65. Kempkes-Matthes B, Walmrath D, Matthes KJ. Protein Z deficiency: a new cause of bleeding tendency. Thromb Res 1995; 79: 49–55.
66. Broze GJJr. Protein Z dependent regulation of coagulation. Thromb Haemost 2001; 86: 8–13.
67. Kemkes-Matthes B, Souri M, Ichinose A, Matthes KJ. First cases of homozygous combined protein Z- and factor V Leiden-mutation in humans. Blood 2000; 96: 74b. Abstract.
68. Kemkes-Matthes B, Nees M, Kuhnel G et al. Protein Z influences prothrombotic phenotype of factor V Leiden in humans. Thromb Res 2002; 106: 183–85.
69. McQuillan AM, Eikelboom JW, Hankey GJ et al. Protein Z in ischemic stroke and its etiologic subtypes. Stroke 2003; 34: 2415–19.
70. Fujimaki K, Yamazaki T, Taniwaki M, Ichinose A. The gene for human protein Z is localized to chromosome 13 at band q34 and is coded by eight regular exons and one alternative exon. Biochemistry 1998; 37: 6838–46.
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