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February 2, 1990, Vol 263, No. 5 >
ARTICLE | February 2, 1990

Protein C and Protein S: Title and subTitle BreakVitamin K—Dependent Inhibitors of Blood Coagulation FREE

Margaret E. Rick, MD
[+] Author Affiliations

Reprint requests to Hematology Service, Bldg 10, Room 2C-390, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892 (Dr Rick).


JAMA. 1990;263(5):701-703. doi:10.1001/jama.1990.03440050095041
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References

SELECTED CASE  A 53-YEAR-OLD asymptomatic white man was evaluated for a possible thrombotic tendency after the death of his 19-year-old son from a pulmonary embolus. The father and his relatives had no history of venous thrombotic disease, but there was a history of coronary artery disease in the family. Physical examination of the father was normal.The deceased son had been athletic and was healthy until he sustained a knee injury that required surgery. His postoperative course had been uneventful for 8 weeks after the surgery, when he died suddenly. The postmortem examination revealed a massive pulmonary embolism. Testing performed on a postmortem blood sample was said to show low protein C levels.Laboratory tests showed that the father had a normal complete blood cell count and platelet count and normal results on screening coagulation tests (prothrombin time, activated partial thromboplastin time, thrombin time, and fibrinogen). Immunologic assays for antithrombin

REFERENCES

1 +
Suttie JW.  Mechanism of action of vitamin K: synthesis of γ-carboxyglutamic acid . CRC Crit Rev Biochem. 1980;;8:191-223.
2 +
Furie B, Furie BC.  The molecular basis of blood coagulation . Cell . 1988;;53:505-518.
3 +
Mann KG, Jenny RJ, Krishnaswamy S.  Cofactor proteins in the assembly and expression of blood clotting enzyme complexes . Annu Rev Biochem. 1988;;57:915-956.
4 +
Esmon CT, Owen WG.  Identification of an endothelial cell cofactor for thrombin-catalyzed activation of protein C . Proc Natl Acad Sci USA. 1981;;78:2249-2252.
5 +
Esmon NL, Owen WG, Esmon CT.  Isolation of a membrane-bound cofactor for thrombin-catalyzed activation of protein C . J Biol Chem. 1982;;257:859-864.
6 +
Walker FJ, Sexton PW, Esmon CT.  The inhibition of blood coagulation by activated protein C through the selective inactivation of activated factor V . Biochim Biophys Acta. 1979;;571:333-342.
7 +
Fulcher CA, Gardner JE, Griffin JH, Zimmerman TS.  Proteolytic inactivation of human factor VIII procoagulant protein by activated protein C and its analogy to factor V . Blood. 1984;;63:486-489.
8 +
Walker FJ, Chavin SI, Fay PJ.  Inactivation of factor VIII by activated protein C and protein S . Arch Biochem Biophys. 1987;;252:322-328.
9 +
Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C.  Deficiency of protein C in congenital thrombotic disease . J Clin Invest. 1981;;68:1370-1373.
10 +
D'Angelo SV, Comp PC, Esmon CT, D'Angelo A.  Relationship between protein C antigen and anticoagulant activity during oral anticoagulation and in selected disease states . J Clin Invest. 1986;;77:416-425.
11 +
Walker FJ.  Regulation of activated protein C by protein S . J Biol Chem. 1981;;256:11128-11131.
12 +
Dahlback B.  Purification of human C4b-binding protein and formation of its complex with vitamin K-dependent protein S . Biochem J. 1983;;209: 847-856.
13 +
Broekmans AW, Veltkamp JJ, Bertina RM.  Congenital protein C deficiency and venous thromboembolism: a study in three Dutch families . N Engl J Med. 1983;;309:340-344.
14 +
Broekmans AW, Bertina RM, Loeliger EA, Hofmann V, Klingemann H-G.  Protein C and the development of skin necrosis during anticoagulant therapy . Thromb Haemost. 1983;;49:251.
15 +
Marciniak E, Wilson HD, Marlar RA.  Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in the blood . Blood. 1984;;65:15-20.
16 +
Mannucci PM, Vigano S.  Deficiencies of protein C, an inhibitor of blood coagulation . Lancet. 1982;;2:463-467.
17 +
McGehee WJ, Klotz TA, Epstein DJ, Rapaport SI.  Coumarin necrosis associated with hereditary protein C deficiency . Ann Intern Med. 1984;;100:59-60.
18 +
Gonzalez R, Alberca I, Sala N, Vicente V.  Protein C deficiency—response to danazol and DDAVP . Thromb Haemost. 1985;;55:320-322.
19 +
Comp PC, Esmon CT.  Recurrent venous thromboembolism in patients with a partial deficiency of protein S . N Engl J Med. 1984;;311:1525-1528.
20 +
Broekmans AW, Bertina RM, Reinalda-Poot J, et al.  Hereditary protein S deficiency and venous thromboembolism—a study of three Dutch families . Thromb Haemost. 1985;;55:273-277.
21 +
Comp PC.  Hereditary disorders predisposing to thrombosis , in Coller BS, ed. Progress in Thrombosis and Hemostais . Orlando, Fla: Grune & Stratton Inc; 1986;;8:71-102.
22 +
Comp PC, Thurnau GR, Welsh J, Esmon CT.  Functional and immunologic protein S levels are decreased during pregnancy . Blood . 1986;;68:881-885.
23 +
Vigano-D'Angelo SV, D'Angelo A, Kaufman CE Jr, Sholer C, Esmon CT, Comp PC.  Protein S deficiency occurs in nephrotic syndrome . Ann Intern Med. 1987;;107:42-47.
24 +
D'Angelo A, Vigano-D'Angelo SV, Esmon CT, Comp PC.  Acquired deficiencies of protein S . J Clin Invest. 1988;;81:1445-1454.
25 +
Miletich J, Sherman L, Broze G Jr.  Absence of thrombosis in subjects with heterozygous protein C deficiency . N Engl J Med. 1987;;317:991-996.

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1 +
Suttie JW.  Mechanism of action of vitamin K: synthesis of γ-carboxyglutamic acid . CRC Crit Rev Biochem. 1980;;8:191-223.
2 +
Furie B, Furie BC.  The molecular basis of blood coagulation . Cell . 1988;;53:505-518.
3 +
Mann KG, Jenny RJ, Krishnaswamy S.  Cofactor proteins in the assembly and expression of blood clotting enzyme complexes . Annu Rev Biochem. 1988;;57:915-956.
4 +
Esmon CT, Owen WG.  Identification of an endothelial cell cofactor for thrombin-catalyzed activation of protein C . Proc Natl Acad Sci USA. 1981;;78:2249-2252.
5 +
Esmon NL, Owen WG, Esmon CT.  Isolation of a membrane-bound cofactor for thrombin-catalyzed activation of protein C . J Biol Chem. 1982;;257:859-864.
6 +
Walker FJ, Sexton PW, Esmon CT.  The inhibition of blood coagulation by activated protein C through the selective inactivation of activated factor V . Biochim Biophys Acta. 1979;;571:333-342.
7 +
Fulcher CA, Gardner JE, Griffin JH, Zimmerman TS.  Proteolytic inactivation of human factor VIII procoagulant protein by activated protein C and its analogy to factor V . Blood. 1984;;63:486-489.
8 +
Walker FJ, Chavin SI, Fay PJ.  Inactivation of factor VIII by activated protein C and protein S . Arch Biochem Biophys. 1987;;252:322-328.
9 +
Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C.  Deficiency of protein C in congenital thrombotic disease . J Clin Invest. 1981;;68:1370-1373.
10 +
D'Angelo SV, Comp PC, Esmon CT, D'Angelo A.  Relationship between protein C antigen and anticoagulant activity during oral anticoagulation and in selected disease states . J Clin Invest. 1986;;77:416-425.
11 +
Walker FJ.  Regulation of activated protein C by protein S . J Biol Chem. 1981;;256:11128-11131.
12 +
Dahlback B.  Purification of human C4b-binding protein and formation of its complex with vitamin K-dependent protein S . Biochem J. 1983;;209: 847-856.
13 +
Broekmans AW, Veltkamp JJ, Bertina RM.  Congenital protein C deficiency and venous thromboembolism: a study in three Dutch families . N Engl J Med. 1983;;309:340-344.
14 +
Broekmans AW, Bertina RM, Loeliger EA, Hofmann V, Klingemann H-G.  Protein C and the development of skin necrosis during anticoagulant therapy . Thromb Haemost. 1983;;49:251.
15 +
Marciniak E, Wilson HD, Marlar RA.  Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in the blood . Blood. 1984;;65:15-20.
16 +
Mannucci PM, Vigano S.  Deficiencies of protein C, an inhibitor of blood coagulation . Lancet. 1982;;2:463-467.
17 +
McGehee WJ, Klotz TA, Epstein DJ, Rapaport SI.  Coumarin necrosis associated with hereditary protein C deficiency . Ann Intern Med. 1984;;100:59-60.
18 +
Gonzalez R, Alberca I, Sala N, Vicente V.  Protein C deficiency—response to danazol and DDAVP . Thromb Haemost. 1985;;55:320-322.
19 +
Comp PC, Esmon CT.  Recurrent venous thromboembolism in patients with a partial deficiency of protein S . N Engl J Med. 1984;;311:1525-1528.
20 +
Broekmans AW, Bertina RM, Reinalda-Poot J, et al.  Hereditary protein S deficiency and venous thromboembolism—a study of three Dutch families . Thromb Haemost. 1985;;55:273-277.
21 +
Comp PC.  Hereditary disorders predisposing to thrombosis , in Coller BS, ed. Progress in Thrombosis and Hemostais . Orlando, Fla: Grune & Stratton Inc; 1986;;8:71-102.
22 +
Comp PC, Thurnau GR, Welsh J, Esmon CT.  Functional and immunologic protein S levels are decreased during pregnancy . Blood . 1986;;68:881-885.
23 +
Vigano-D'Angelo SV, D'Angelo A, Kaufman CE Jr, Sholer C, Esmon CT, Comp PC.  Protein S deficiency occurs in nephrotic syndrome . Ann Intern Med. 1987;;107:42-47.
24 +
D'Angelo A, Vigano-D'Angelo SV, Esmon CT, Comp PC.  Acquired deficiencies of protein S . J Clin Invest. 1988;;81:1445-1454.
25 +
Miletich J, Sherman L, Broze G Jr.  Absence of thrombosis in subjects with heterozygous protein C deficiency . N Engl J Med. 1987;;317:991-996.

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