Abstract

The coagulation proteins, Factor VIII/von Willebrand Factor and Factor IX will be isoelectric focused directly in plasma. They will then be identified with 125I labeled affinity purified antibodies. Different isoelectric patterns of Factor VIII/von Willebrand Factors will be used to classify different types of von Willebrand's disease. The function of different isoelectric forms of Factor VIII/von Willebrand Factor will be determined. Similarly, isoelectric patterns of Factor IX will be used to classify different types of hemophilia B. Following their isolation, the structural and functional relationships of different Factor IX forms will be explored.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Project (R01)
Project #
2R01HL015491-13
Application #
3334963
Study Section
(EH)
Project Start
1976-05-01
Project End
1986-04-30
Budget Start
1985-05-01
Budget End
1986-04-30
Support Year
13
Fiscal Year
1985
Total Cost
Indirect Cost

  Institution

Name
Scripps Research Institute
Department
Type
DUNS #
City
San Diego
State
CA
Country
United States
Zip Code
92037

  Publications

Ikeda, Y; Handa, M; Kawano, K et al. (1991) The role of von Willebrand factor and fibrinogen in platelet aggregation under varying shear stress. J Clin Invest 87:1234-40
Sugimoto, M; Ricca, G; Hrinda, M E et al. (1991) Functional modulation of the isolated glycoprotein Ib binding domain of von Willebrand factor expressed in Escherichia coli. Biochemistry 30:5202-9
Sugimoto, M; Mohri, H; McClintock, R A et al. (1991) Identification of discontinuous von Willebrand factor sequences involved in complex formation with botrocetin. A model for the regulation of von Willebrand factor binding to platelet glycoprotein Ib. J Biol Chem 266:18172-8
Fujimura, Y; Titani, K; Usami, Y et al. (1991) Isolation and chemical characterization of two structurally and functionally distinct forms of botrocetin, the platelet coagglutinin isolated from the venom of Bothrops jararaca. Biochemistry 30:1957-64
Dent, J A; Galbusera, M; Ruggeri, Z M (1991) Heterogeneity of plasma von Willebrand factor multimers resulting from proteolysis of the constituent subunit. J Clin Invest 88:774-82
Ware, J; Dent, J A; Azuma, H et al. (1991) Identification of a point mutation in type IIB von Willebrand disease illustrating the regulation of von Willebrand factor affinity for the platelet membrane glycoprotein Ib-IX receptor. Proc Natl Acad Sci U S A 88:2946-50
De Marco, L; Mazzucato, M; De Roia, D et al. (1990) Distinct abnormalities in the interaction of purified types IIA and IIB von Willebrand factor with the two platelet binding sites, glycoprotein complexes Ib-IX and IIb-IIIa. J Clin Invest 86:785-92
Dent, J A; Berkowitz, S D; Ware, J et al. (1990) Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. Proc Natl Acad Sci U S A 87:6306-10
Vicente, V; Houghten, R A; Ruggeri, Z M (1990) Identification of a site in the alpha chain of platelet glycoprotein Ib that participates in von Willebrand factor binding. J Biol Chem 265:274-80
Mohri, H; Yoshioka, A; Zimmerman, T S et al. (1989) Isolation of the von Willebrand factor domain interacting with platelet glycoprotein Ib, heparin, and collagen and characterization of its three distinct functional sites. J Biol Chem 264:17361-7

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