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Deficiency of protein C in congenital thrombotic disease.
Open accessAbstract
A family with a history of recurring thrombosis was studied to determine if a plasma protein deficiency could account for the observed disease. Protein C levels in plasma were determined immunologically using the Laurell rocket technique. The propositus, his father, and his paternal uncle, who are severely affected, had 38-49% of normal levels of protein C antigen, whereas unaffected family members had normal levels. There was no familial deficiency of antithrombin III and plasminogen. Because activated protein C is a potent in vitro anticoagulant enzyme and an in vivo profibrinolytic agent, it is suggested that the recurrent thrombotic disease in this family is due to an inherited deficiency in protein C.
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Cited by
Recurrent Venous Thromboembolism in Patients with a Partial Deficiency of Protein S
Comp, P. C., & Esmon, C. T. (1984) · New England Journal of Medicine · doi:10.1056/nejm198412133112401 · Cited by 508
Heijboer, H., Brandjes, D. P. M., Büller, H. R., Sturk, A., & ten Cate, J. W. (1990) · New England Journal of Medicine · doi:10.1056/nejm199011293232202 · Cited by 445
Rodgers, G., & Conn, M. (1990) · Blood · doi:10.1182/blood.v75.4.895.895 · Cited by 398
Increased risk of venous thrombosis in carriers of hereditary protein C deficiency defect
Allaart, C. F., Poort, S. R., Reitsma, P. H., Bertina, R. M., Briët, E., Rosendaal, F. R., & Rosendaal, F. R. (1993) · The Lancet · doi:10.1016/0140-6736(93)90003-y · Cited by 351
Branson, H., Marble, R., Katz, J., & Griffin, J. (1983) · The Lancet · doi:10.1016/s0140-6736(83)91216-3 · Cited by 342
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Metadata
- Authors
- Griffin, J. H., Evatt, B., Zimmerman, T. S., Kleiss, A. J., & Wideman, C.
- Venue
- Journal of Clinical Investigation
- Published
- 1981-11-01
- Volume
- 68
- Issue
- 5
- Pages
- 1370-1373
- ISSN
- 0021-9738 (ISSN-L)
- OpenAlex
- W2085445739
- Cited by
- 1,250
- References
- 0
- FWCI
- 22.05
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Griffin, J. H., Evatt, B., Zimmerman, T. S., Kleiss, A. J., & Wideman, C. (1981). Deficiency of protein C in congenital thrombotic disease. Journal of Clinical Investigation. https://doi.org/10.1172/jci110385
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