|
|
 Previous Article | Table of Contents | Next Article 
An abnormal plasma distribution of protein S occurs in functional protein S
deficiency
PC Comp, D Doray, D Patton and CT Esmon
Protein S is a natural anticoagulant present in the plasma that serves as a
cofactor for activated protein C. Patients deficient in protein S are
subject to recurrent venous thrombotic disease. Protein S deficiency
differs from other plasma protein deficiencies in that deficient patients
often have normal or only mildly reduced levels of protein S in their
plasma as detected by conventional immunologic methods but have markedly
reduced functional protein S levels. This apparent discrepancy is due to
the presence of two forms of protein S in plasma. The protein S is present
free and in a complex with C4b- binding protein. The free form is
functionally active, whereas the bound form is not. Examination by crossed
immunoelectrophoresis of 31 functionally protein S-deficient individuals
from seven families reveals that 29 of the 31 have all or most of their
protein S complexed to C4b-binding protein with little or no free protein
and have correspondingly low levels of protein S functional activity (type
I deficiency). Two related protein S-deficient individuals show a different
type of distribution with little or no protein S, either bound or free
(type II deficiency). The detection and classification of protein
S-deficient individuals requires the application of both a functional assay
and an assessment of protein S distribution between bound and free forms.
Volume 67,
Issue 2,
pp. 504-508,
02/01/1986
Copyright © 1986 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
K. A. Archer, T. Lembo Jr., and J. A. Haber
Protein S Deficiency and Lower-Extremity Arterial Thrombosis: Complicating a Common Presentation
J Am Podiatr Med Assoc,
March 1, 2007;
97(2):
151 - 155.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Harada, H. Morita, Y. Imai, D. Hayashi, N. Takeda, K. Maemura, Y. Yazaki, and R. Nagai
Deficiency of Protein S-Mediated Familial Venous Thrombophilia: A Case Report
Angiology,
May 1, 2003;
54(3):
377 - 381.
[Abstract]
[PDF]
|
|
|
|
|
|
|
I. Martinelli, E. Taioli, P. Bucciarelli, S. Akhavan, and P. M. Mannucci
Interaction Between the G20210A Mutation of the Prothrombin Gene and Oral Contraceptive Use in Deep Vein Thrombosis
Arterioscler. Thromb. Vasc. Biol.,
March 1, 1999;
19(3):
700 - 703.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
I. Martinelli, P. M. Mannucci, V. De Stefano, E. Taioli, V. Rossi, F. Crosti, K. Paciaroni, G. Leone, and E. M. Faioni
Different Risks of Thrombosis in Four Coagulation Defects Associated With Inherited Thrombophilia: A Study of 150 Families
Blood,
October 1, 1998;
92(7):
2353 - 2358.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
I. Martinelli, E. Sacchi, G. Landi, E. Taioli, F. Duca, and P. M. Mannucci
High Risk of Cerebral-Vein Thrombosis in Carriers of a Prothrombin-Gene Mutation and in Users of Oral Contraceptives
N. Engl. J. Med.,
June 18, 1998;
338(25):
1793 - 1797.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. L. Bick and H. Kaplan
Syndromes of Thrombosis and Hypercoagulability: Congenital and Acquired Thrombophilias
Clinical and Applied Thrombosis/Hemostasis,
January 1, 1998;
4(1):
25 - 50.
[Abstract]
[PDF]
|
|
|
|
|
|
|
H. PIERRE-JACQUES, C. J. GLUECK, M. A. MONT, and D. S. HUNGERFORD
Familial Heterozygous Protein-S Deficiency in a Patient Who Had Multifocal Osteonecrosis. A Case Report
J. Bone Joint Surg. Am.,
July 1, 1997;
79(7):
1079 - 84.
[Full Text]
|
|
|
|
|
|
|
T. Atsumi, M. Khamashta, P. Ames, K. Ichikawa, T. Koike, and G. Hughes
Effect of {beta}2glycoprotein I and human monoclonal anticardiolipin antibody on the protein S/C4b-binding protein system
Lupus,
January 1, 1997;
6(4):
358 - 364.
[Abstract]
[PDF]
|
|
|
|
|
|
|
M. Li and G. L. Long
Identification of Two Novel Point Mutations in the Human Protein S Gene Associated With Familial Protein S Deficiency and Thrombosis
Arterioscler. Thromb. Vasc. Biol.,
December 1, 1996;
16(12):
1407 - 1415.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
R. F. Macko, S. F. Ameriso, A. Gruber, J. H. Griffin, J. A. Fernandez, R. Barndt, F. P. Quismorio, J. M. Weiner, and M. Fisher
Impairments of the Protein C System and Fibrinolysis in Infection-Associated Stroke
Stroke,
November 1, 1996;
27(11):
2005 - 2011.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
S. Hirokawa and E. F. Mammen
A Functional Protein S and Microlatex Immunoassay for Protein S and C4b-Binding Protein on the Automated Coagulation Laboratory (ACL) 300 Plus
Clinical and Applied Thrombosis/Hemostasis,
October 1, 1996;
2(4):
268 - 275.
[Abstract]
[PDF]
|
|
|
|
|
|
|
I. Pabinger and B. Schneider
Thrombotic Risk in Hereditary Antithrombin III, Protein C, or Protein S Deficiency : A Cooperative, Retrospective Study
Arterioscler. Thromb. Vasc. Biol.,
June 1, 1996;
16(6):
742 - 748.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
A. D'Angelo, P. D. Valle, L. Crippa, E. Pattarini, L. Grimaldi, and S. V. D'Angelo
Autoimmune Protein S Deficiency in a Boy with Severe Thromboembolic Disease
N. Engl. J. Med.,
June 17, 1993;
328(24):
1753 - 1757.
[Full Text]
|
|
|
|
|
