|
|
 Previous Article | Table of Contents | Next Article 
Blood coagulation factor Va abnormality associated with resistance to
activated protein C in venous thrombophilia
X Sun, B Evatt and JH Griffin
Department of Molecular and Experimental Medicine, Scripps Research
Institute, La Jolla, CA 92037.
A coagulation test abnormality, termed activated protein C (APC)
resistance, involving poor anticoagulant response to APC is currently the
most common laboratory finding among venous thrombophilic patients. Because
the anticoagulant activity of APC involves inactivation of factors Va and
VIIIa, studies were made to assess the presence of abnormal factors V or
VIII. Diluted aliquots of plasma from two unrelated patients with APC
resistance and thrombosis were added to either factor VIII-deficient or
factor V-deficient plasma and APC resistance assays were performed. The
results suggested that patients' factor V but not factor VIII rendered the
substrate plasma APC resistant. When factor V that had been partially
purified from normal or APC resistant patients' plasmas using
immunoaffinity chromatography was added to factor V-deficient plasma, APC
resistance assays showed that patients' factor V or factor Va, but not
normal factor V, rendered the substrate plasma resistant to APC. Studies of
the inactivation of each partially purified thrombin-activated factor Va by
APC suggested that half of the patients' factor Va was resistant to APC.
These results support the hypothesis that the APC resistance of some venous
thrombophilic plasmas is caused by abnormal factor Va.
Volume 83,
Issue 11,
pp. 3120-3125,
06/01/1994
Copyright © 1994 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:
|
|
|
|
 
B. Dahlback
Advances in understanding pathogenic mechanisms of thrombophilic disorders
Blood,
July 1, 2008;
112(1):
19 - 27.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. G. Mann and M. Kalafatis
Factor V: a combination of Dr Jekyll and Mr Hyde
Blood,
January 1, 2003;
101(1):
20 - 30.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. R. Lentz, D. J. Piegors, J. A. Fernandez, R. A. Erger, E. Arning, M. R. Malinow, J. H. Griffin, T. Bottiglieri, W. G. Haynes, and D. D. Heistad
Effect of hyperhomocysteinemia on protein C activation and activity
Blood,
August 28, 2002;
100(6):
2108 - 2112.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. Deguchi, J. A. Fernandez, and J. H. Griffin
Neutral Glycosphingolipid-dependent Inactivation of Coagulation Factor Va by Activated Protein C and Protein S
J. Biol. Chem.,
March 8, 2002;
277(11):
8861 - 8865.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Strater, K. Kurnik, C. Heller, R. Schobess, P. Luigs, and U. Nowak-Gottl
Aspirin Versus Low-Dose Low-Molecular-Weight Heparin: Antithrombotic Therapy in Pediatric Ischemic Stroke Patients: A Prospective Follow-Up Study
Stroke,
November 1, 2001;
32(11):
2554 - 2558.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P Deltenre, M-H Denninger, S Hillaire, M-C Guillin, N Casadevall, J Briere, S Erlinger, and D-C Valla
Factor V Leiden related Budd-Chiari syndrome
Gut,
February 1, 2001;
48(2):
264 - 268.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Gunther, R. Junker, R. Strater, R. Schobess, K. Kurnik, A. Kosch, U. Nowak-Gottl, and f. t. C. S. S. Group
Symptomatic Ischemic Stroke in Full-Term Neonates : Role of Acquired and Genetic Prothrombotic Risk Factors
Stroke,
October 1, 2000;
31(10):
2437 - 2441.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Kowalski, D. Radu, and B. Gold
Colorimetric Microwell Plate Detection of the Factor V Leiden Mutation
Clin. Chem.,
August 1, 2000;
46(8):
1195 - 1198.
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. Rodeghiero and A. Tosetto
Activated Protein C Resistance and Factor V Leiden Mutation Are Independent Risk Factors for Venous Thromboembolism
Ann Intern Med,
April 20, 1999;
130(8):
643 - 650.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Thorelli, R. J. Kaufman, and B. Dahlback
Cleavage of Factor V at Arg 506 by Activated Protein C and the Expression of Anticoagulant Activity of Factor V
Blood,
April 15, 1999;
93(8):
2552 - 2558.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. O. Yildiz, B. Oran, Y. Buyukasik, b. C. Haznedaroglu, S. Cekirge, and S. Kirazli
Acute Budd-Chiari Syndrome Associated with Activated Protein C Resistance During Intravenous Anticoagulant Treatment
Clinical and Applied Thrombosis/Hemostasis,
July 1, 1998;
4(3):
220 - 223.
[Abstract]
[PDF]
|
|
|
|
|
|
|
T. L. Yang, J. Cui, A. Rehumtulla, A. Yang, M. Moussalli, R. J. Kaufman, and D. Ginsburg
The Structure and Function of Murine Factor V and Its Inactivation by Protein C
Blood,
June 15, 1998;
91(12):
4593 - 4599.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Z. Laszik, A. Mitro, F. B. Taylor Jr, G. Ferrell, and C. T. Esmon
Human Protein C Receptor Is Present Primarily on Endothelium of Large Blood Vessels : Implications for the Control of the Protein C Pathway
Circulation,
November 18, 1997;
96(10):
3633 - 3640.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
C. Maher, C. Wall, and S. Fanning
Molecular Genetics of Factor V Leiden: Genetic Origins and Modern DNA-Based Detection Strategies
Seminars in Cardiothoracic and Vascular Anesthesia,
November 1, 1997;
1(4):
333 - 341.
[Abstract]
[PDF]
|
|
|
|
|
|
|
J. A. Fernandez, T. M. Hackeng, K. Kojima, and J. H. Griffin
The Carbohydrate Moiety of Factor V Modulates Inactivation by Activated Protein C
Blood,
June 15, 1997;
89(12):
4348 - 4354.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Kalafatis and K. G. Mann
Factor VLeiden and Thrombophilia
Arterioscler Thromb Vasc Biol,
April 1, 1997;
17(4):
620 - 627.
[Full Text]
|
|
|
|
|
|
|
A. Zivelin, J. H. Griffin, X. Xu, I. Pabinger, M. Samama, J. Conard, B. Brenner, A. Eldor, and U. Seligsohn
A Single Genetic Origin for a Common Caucasian Risk Factor for Venous Thrombosis
Blood,
January 15, 1997;
89(2):
397 - 402.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Zoller, A. Hillarp, and B. Dahlback
State-of-the-Art Review: Activated Protein C Resistance: Clinical Implications
Clinical and Applied Thrombosis/Hemostasis,
January 1, 1997;
3(1):
25 - 32.
[Abstract]
[PDF]
|
|
|
|
|
|
|
A. Bengtsson, B. Zoller, P. de Frutos, B. Dahlback, and G. Sturfelt
Factor V:Q506 mutation and anticardiolipin antibodies in systemic lupus erythematosus
Lupus,
December 1, 1996;
5(6):
598 - 601.
[Abstract]
[PDF]
|
|
|
|
|
|
|
M. Fisher, J. A. Fernandez, S. F. Ameriso, D. Xie, A. Gruber, A. Paganini-Hill, and J. H. Griffin
Activated Protein C Resistance in Ischemic Stroke Not Due to Factor V Arginine506->Glutamine Mutation
Stroke,
July 1, 1996;
27(7):
1163 - 1166.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
K.A. Bauer and F.M. Graeme-Cook
Case 12-1996- An 18-year-old woman with hepatomegaly and ascites
N. Engl. J. Med.,
April 18, 1996;
334(16):
1045 - 1051.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Kalafatis, D. Lu, R. M. Bertina, G. L. Long, and K. G. Mann
Biochemical Prototype for Familial Thrombosis : A Study Combining a Functional Protein C Mutation and Factor V Leiden
Arterioscler Thromb Vasc Biol,
December 1, 1995;
15(12):
2181 - 2187.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
M. Kalafatis, R. M. Bertina, M. D. Rand, and K. G. Mann
Characterization of the Molecular Defect in Factor V[IMAGE]
J. Biol. Chem.,
February 24, 1995;
270(8):
4053 - 4057.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. S. Greengard, S. Eichinger, J. H. Griffin, and K. A. Bauer
Variability of Thrombosis among Homozygous Siblings with Resistance to Activated Protein C Due to an Arg-to-Gln Mutation in the Gene for Factor V
N. Engl. J. Med.,
December 8, 1994;
331(23):
1559 - 1562.
[Full Text]
|
|
|
|
|
|
|
Z.-F. Yin, Z.-F. Huang, J. Cui, R. Fiehler, N. Lasky, D. Ginsburg, and G. J. Broze Jr.
Prothrombotic phenotype of protein Z deficiency
PNAS,
June 6, 2000;
97(12):
6734 - 6738.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
