Deletion of P1 arginine in a novel antithrombin variant (antithrombin London) abolishes inhibitory activity but enhances heparin affinity and is associated with early onset thrombosis

Journal of Biological Chemistry

Volumn 278, Issue 16, 2003, Pages 13688-13695

  Raja, Srikumar M ^a   Chhablani, Neetu ^a   Swanson, Richard ^a   Thompson, Elizabeth ^b   Laffan, Mike ^b   Lane, David A ^b   Olson, Steven T ^a  

^a UNIVERSITY OF ILLINOIS AT CHICAGO   (United States)

^b IMPERIAL COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

BODY FLUIDS; CATALYSIS; CHEMICAL ACTIVATION; MOLECULAR DYNAMICS;

ANTITHROMBIN;

ENZYME INHIBITION;

ANTITHROMBIN; ANTITHROMBIN LONDON; ARGININE; HEPARAN SULFATE; HEPARIN; SERINE PROTEINASE INHIBITOR; UNCLASSIFIED DRUG; BLOOD CLOTTING FACTOR 10A; PROTEIN; THROMBIN;

AMINO ACID SUBSTITUTION; ARTICLE; BINDING AFFINITY; BLOOD CLOTTING; CLINICAL ARTICLE; CODON; FEMALE; FLOW KINETICS; FRAMESHIFT MUTATION; HOMEOSTASIS; HUMAN; MALE; MOLECULAR STABILITY; POLYACRYLAMIDE GEL ELECTROPHORESIS; PRIORITY JOURNAL; PROTEIN FAMILY; PROTEIN FUNCTION; PROTEIN PURIFICATION; THROMBOGENESIS; THROMBOSIS; AFFINITY CHROMATOGRAPHY; BINDING COMPETITION; BIOLOGICAL MODEL; CHEMISTRY; DRUG ANTAGONISM; FAMILY HEALTH; GENE DELETION; GENETICS; HETEROZYGOTE; KINETICS; LIQUID CHROMATOGRAPHY; METABOLISM; ONSET AGE; PEDIGREE; PROTEIN BINDING; TIME;

AGE OF ONSET; ANTITHROMBINS; ARGININE; BINDING, COMPETITIVE; BLOOD COAGULATION; CHROMATOGRAPHY, AFFINITY; CHROMATOGRAPHY, LIQUID; ELECTROPHORESIS, POLYACRYLAMIDE GEL; FACTOR XA; FAMILY HEALTH; FEMALE; GENE DELETION; HEPARIN; HEPARITIN SULFATE; HETEROZYGOTE; HUMANS; KINETICS; MALE; MODELS, BIOLOGICAL; PEDIGREE; PROTEIN BINDING; PROTEINS; THROMBIN; THROMBOSIS; TIME FACTORS;

EID: 0038190864     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.M300062200     Document Type: Article

References (35)

1. Björk, I., and Olson, S. T. (1997) in Chemistry and Biology of Serpins (Church, F. C., Cunningham, D. D., Ginsburg, D., Hoffman, M., Stone, S. R., and Tollefsen, D. M., eds) pp. 17-33, Plenum Press, New York
2. van Boven, H. H., and Lane, D. A. (1997) Semin. Hematol. 34, 188-204
3. Ishiguro, K., Kojima, T., Kadomatsu, K., Nakayama, Y., Takagi, A., Suzuki, M., Takeda, N., Ito, M., Yamamoto, K., Matsushita, T., Kusugami, K., Muramatsu, T., and Saito, H. (2000) J. Clin. Invest. 106, 873-878
4. Thunberg, L., Bäckström, G., and Lindahl, U. (1982) Carbohydr. Res. 100, 393-410
5. Choay, J., Petitou, M., Lormeau, J. C., Sinaÿ, P., Casu, B., and Gatti, G. (1983) Biochem. Biophys. Res. Commun. 116, 492-499
6. Olson, S. T., Srinivasan, K. R., Björk, I., and Shore, J. D. (1981) J. Biol. Chem. 256, 11073-11079
7. Olson, S. T., Björk, I., Sheffer, R., Craig, P. A., Shore, J. D., and Choay, J. (1992) J. Biol. Chem. 267, 12528-12538
8. Marcum, J. A., Atha, D. H., Fritze, L. M. S., Nawroth, P. P., Stern, D. M., and Rosenberg, R. D. (1986) J. Biol. Chem. 261, 7507-7517
9. Skinner, R., Abrahams, J.-P., Whisstock, J. C., Lesk, A. M., Carrell, R. W., and Wardell, M. R. (1997) J. Mol. Biol. 266, 601-609
10. Jin, L., Abrahams, J. P., Skinner, R., Petitou, M., Pike, R. N., and Carrell, R. W. (1997) Proc. Natl. Acad. Sci., U. S. A. 94, 14683-14688
11. Chuang, Y.-J., Swanson, R., Raja, S. M., and Olson, S. T. (2001) J. Biol. Chem. 276, 14961-14971
12. Sas, G. D., Pepper, D. S., and Cash, J. D. (1975) Thromb. Res. 6, 87-94
13. Lane, D. A., Olds, R., Conard, J., Boisclair, M., Bock, S. C., Hultin, M., Abildgaard, U., Ireland, H., Thompson, E., Sas, G., Horellou, M. H., Tamponi, G., and Thein, S. L. (1992) J. Clin. Invest. 90, 2422-2433
14. Chowdhury, V., Olds, R. J., Lane, D. L., Pabinger, I., Machin, S. J., Ryan, K., Bauer, K. A., Bhavani, M., Abildgaard, U., Finazzi, G., Conard, J., Castaman, G., Mannucci, P., and Thein, S. L. (1993) Br. J. Haematol. 84, 656-661
15. Olson, S. T., Björk, I., and Shore, J. D. (1993) Methods Enzymol. 222, 525-560
16. Nordenman, B., Nyström, C., and Björk, I. (1977) Eur. J. Biochem. 78, 195-203
17. van Boeckel, C. A. A., and Petitou, M. (1993) Angew. Chem. Int. Ed. Engl. 32, 1671-1690
18. Miletich, J. P., Broze, G. J., Jr., and Majerus, P. W. (1981) Methods Enzymol. 80, 221-228
19. Owen, W. G., and Jackson, C. M. (1973) Thromb. Res. 3, 705-714
20. Chuang, Y.-J., Swanson, R., Raja, S. M., Bock, S. C., and Olson, S. T. (2001) Biochemistry 40, 6670-6679
21. Laemmli, U. K. (1970) Nature 227, 680-685
22. Turk, B., Brieditis, I., Bock, S. C., Olson, S. T., and Björk, I. (1997) Biochemistry 36, 6682-6691
23. Lindahl, P., Raub-Segall, E., Olson, S. T., and Björk, I. (1991) Biochem. J. 276, 387-394
24. Olson, S. T., Stephens, A. W., Hirs, C. H. W., Bock, P. E., and Björk, I. (1995) J. Biol. Chem. 270, 9717-9724
25. Wardell, M. R., Chang, W. S. W., Bruce, D., Skinner, R., Lesk, A. M., and Carrell, R. W. (1997) Biochemistry 36, 13133-13142
26. Erdjument, H., Lane, D. A., Panico, M., DiMarzo, V., and Morris, H. R. (1988) J. Biol. Chem. 263, 5589-5593
27. Lane, D. A., Erdjument, H., Thompson, E., Panico, M., DiMarzo, V., Morris, H. R., Leone, G., DeStefano, V., and Thein, S. L. (1989) J. Biol. Chem. 264, 10200-10204.
28. Björk, I., Jackson, C. M., Jornvall, H., Lavine, K. K., Nordling, K., and Salsgiver, W. J. (1982) J. Biol. Chem. 257, 2406-2411
29. Jordan, R. E., Oosta, G. M., Gardner, W. T., and Rosenberg, R. D. (1980) J. Biol. Chem. 255, 10081-10090
30. Desai, U. R., Petitou, M., Björk, I., and Olson, S. T. (1998) J. Biol. Chem. 273, 7478-7487
31. Desai, U. R., Petitou, M., Björk, I., and Olson, S. T. (1998) Biochemistry 37, 13033-13041
32. Futamura, A., and Gettins, P. G. W. (2000) J. Biol. Chem. 275, 4092-4098
33. Huntington, J. A., Olson, S. T., Fan, B., and Gettins, P. G. W. (1996) Biochemistry 35, 8495-8503
34. Rezaie, A. R. (2002) J. Biol. Chem. 277, 1235-1239
35. van Boven, H. H., Vandenbroucke, J. P., Briet, E., and Rosendall, F. R. (1999) Blood 94, 2590-2594