Ebook: Recombinant Technology in Hemostasis and Thrombosis

Recent progress in molecular biology has led to a rapid expansion of our understanding of the proteins that are essential for hemostasis and thrombosis. The goal of the XXI Annual Scientific Symposium of the American Red Cross was to provide a forum to explore and document the impact of recombinant DNA technology in this field. The speakers described the essential features of the genes responsible for key plasma proteins important in hemostasis, including procoagulant Factors VIII and IX and anticoagulant proteins, Antithrombin III and Protein C. They emphasized the advances in recombinant DNA technology that have led to the cloning of these genes. Careful examination of the gene sequence has then provided a clearer understanding of the structure of the encoded proteins, and has given additional insight into their functional domains and their interactions in hemostasis. At the same time, these advances have made it possible to better characterize hemostatic disorders. A large number of published studies have shown that the mutations affecting biological activity are clustered in areas that define functional domains. They have led to fundamental advances in our understanding of specific diseases and they have made it possible to develop more accurate and sensitive diagnostic tests for the detection of the disease states.

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Content:
Front Matter....Pages i-viii
Front Matter....Pages 1-1
Biosynthesis and Assembly of the Factor VIII-Von Willebrand Factor Complex....Pages 3-12
Factor IX: Gene Structure and Protein Synthesis....Pages 13-24
Antithrombin III Genetics, Structure and Function....Pages 25-45
Interactions Between The Functional Domains of Antithrombin III....Pages 47-63
Protein C: Gene Structure and Protein Synthesis....Pages 65-78
Structural and Functional Properties of Protein C....Pages 79-98
Front Matter....Pages 99-99
Molecular Defects in Hemophilia A....Pages 101-113
Molecular Defects in Hemophilia B....Pages 115-131
Molecular Defects in Human Antithrombin III Deficiency....Pages 133-146
The Biologic Impact of Hereditary Defects that Cause Thrombosis....Pages 147-167
Front Matter....Pages 169-169
Factors Limiting Expression of Secreted Proteins in Mammalian Cells....Pages 171-185
Synthesis of Biologically Active Vitamin K-Dependent Coagulation Factors....Pages 187-195
The Expression of Therapeutic Proteins in Transgenic Animals....Pages 197-209
Front Matter....Pages 211-211
The Use of Animal Models to Evaluate Proteins Produced by Recombinant Technology....Pages 213-221
Experiences with Recombinant Factor VIIa in Hemophiliacs....Pages 223-228
Clinical Trials of Factor VIII Produced by Recombinant Technology....Pages 229-233
Clinical Trials of Recombinant Factor VIII....Pages 235-241
Front Matter....Pages 243-243
The Impact of Recombinant Technologies in Understanding Plasma Proteins Important for Hemostasis and Thrombosis....Pages 245-249
Back Matter....Pages 251-261

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Content:
Front Matter....Pages i-viii
Front Matter....Pages 1-1
Biosynthesis and Assembly of the Factor VIII-Von Willebrand Factor Complex....Pages 3-12
Factor IX: Gene Structure and Protein Synthesis....Pages 13-24
Antithrombin III Genetics, Structure and Function....Pages 25-45
Interactions Between The Functional Domains of Antithrombin III....Pages 47-63
Protein C: Gene Structure and Protein Synthesis....Pages 65-78
Structural and Functional Properties of Protein C....Pages 79-98
Front Matter....Pages 99-99
Molecular Defects in Hemophilia A....Pages 101-113
Molecular Defects in Hemophilia B....Pages 115-131
Molecular Defects in Human Antithrombin III Deficiency....Pages 133-146
The Biologic Impact of Hereditary Defects that Cause Thrombosis....Pages 147-167
Front Matter....Pages 169-169
Factors Limiting Expression of Secreted Proteins in Mammalian Cells....Pages 171-185
Synthesis of Biologically Active Vitamin K-Dependent Coagulation Factors....Pages 187-195
The Expression of Therapeutic Proteins in Transgenic Animals....Pages 197-209
Front Matter....Pages 211-211
The Use of Animal Models to Evaluate Proteins Produced by Recombinant Technology....Pages 213-221
Experiences with Recombinant Factor VIIa in Hemophiliacs....Pages 223-228
Clinical Trials of Factor VIII Produced by Recombinant Technology....Pages 229-233
Clinical Trials of Recombinant Factor VIII....Pages 235-241
Front Matter....Pages 243-243
The Impact of Recombinant Technologies in Understanding Plasma Proteins Important for Hemostasis and Thrombosis....Pages 245-249
Back Matter....Pages 251-261
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