The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.
Plasma protein S deficiency in familial thrombotic disease
Abstract
A family with a history of severe recurrent venous thromboembolic disease was studied to determine if a plasma protein deficiency could account for observed disease. Protein S levels in plasma were determined immunologically using the Laurell rocket technique. The propositus, his mother, his aunt, and his cousin who were clinically affected had 17% to 65% of the control levels of protein S antigen (normal range, 71% to 147%). Since three of these patients were receiving oral anticoagulant therapy, the ratios of protein S to prothrombin, factor X, and protein C in these patients were compared with values for a group of orally anticoagulated controls. These results suggested that protein S is half-normal in all family members with thrombotic disease. Other proteins known to be associated with familial thrombotic disease, including antithrombin III, plasminogen, fibrinogen, and protein C, were normal. Because plasma protein S serves as a cofactor for the anticoagulant activity of activated protein C and because protein C deficiency is associated with recurrent thrombotic disease, it is suggested that recurrent thrombotic disease in this family is the result of an inherited deficiency of protein S.
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Citations
Cites
PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT
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Deficiency of protein C in congenital thrombotic disease.
Griffin, J. H., Evatt, B., Zimmerman, T. S., Kleiss, A. J., & Wideman, C. (1981) · Journal of Clinical Investigation · doi:10.1172/jci110385 · Cited by 1,250
Self-citationInherited Antithrombin Deficiency Causing Thrombophilia
Egeberg, O. (1965) · Thrombosis and Haemostasis · doi:10.1055/s-0038-1656297 · Cited by 1,030
Di Scipio, R. G., Hermodson, M. A., Yates, S. G., & Davie, E. W. (1977) · Biochemistry · doi:10.1021/bi00623a022 · Cited by 579
Regulation of activated protein C by a new protein. A possible function for bovine protein S.
Walker, F. J. (1980) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)70660-7 · Cited by 470
Congenital Protein C Deficiency and Venous Thromboembolism
Broekmans, A. W., Veltkamp, J. J., & Bertina, R. M. (1983) · New England Journal of Medicine · doi:10.1056/nejm198308113090604 · Cited by 409
DiScipio, R. G., & Davie, E. W. (1979) · Biochemistry · doi:10.1021/bi00572a026 · Cited by 255
Purification of human vitamin K-dependent protein S and its limited proteolysis by thrombin
Dahlbäck, B. (1983) · Biochemical Journal · doi:10.1042/bj2090837 · Cited by 182
Cited by
Poort, Sr., Rosendaal, F., Reitsma, P., & Bertina, R. (1996) · Blood · doi:10.1182/blood.v88.10.3698.bloodjournal88103698 · Cited by 3,079
Oosting, J., Derksen, R., Bobbink, I., Hackeng, T., Bouma, B., & de Groot, P. (1993) · Blood · doi:10.1182/blood.v81.10.2618.2618 · Cited by 488
Anticoagulant protein C pathway defective in majority of thrombophilic patients [see comments]
Griffin, J., Evatt, B., Wideman, C., & Fernandez, J. (1993) · Blood · doi:10.1182/blood.v82.7.1989.1989 · Cited by 417
Self-citationRosing, J., Tans, G., Nicolaes, G. A. F., Thomassen, M. C. L. G. D., Van Oerle, R., Van DerPloeg, P. M. E. N., Heijnen, P., Hamulyak, K., & Hemker, H. C. (1997) · British Journal of Haematology · doi:10.1046/j.1365-2141.1997.192707.x · Cited by 325
Elevated Factor VIII Levels and the Risk of Thrombosis
Kamphuisen, P. W., Eikenboom, J. C. J., & Bertina, R. M. (2001) · Arteriosclerosis, Thrombosis, and Vascular Biology · doi:10.1161/01.atv.21.5.731 · Cited by 316
Metadata
- Authors
- Schwarz, H., Fischer, M., Hopmeier, P., Batard, M., & Griffin, J.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 1984-12-01
- Volume
- 64
- Issue
- 6
- Pages
- 1297-1300
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W362194484
- Cited by
- 526
- References
- 8
- FWCI
- 9.98
- Type
- journal article
- Language
- en
- Open access
- closed
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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