Factor VIII Arg2304→ his binds to phosphatidylserine and is a functional cofactor in the factor X-ase complex

Thrombosis and Haemostasis

Volumn 85, Issue 2, 2001, Pages 260-264

  Lewis, D A ^a   Moore, K D ^a   Ortel, T L ^a  

^a DUKE UNIVERSITY   (United States)

Author keywords

Factor VIII; Factor VIII light chain; Hemophilia; Phospholipid binding

Indexed keywords

BLOOD CLOTTING FACTOR 10; BLOOD CLOTTING FACTOR 8; PHOSPHATIDYLSERINE; CANCER PROCOAGULANT; CYSTEINE PROTEINASE; TUMOR PROTEIN;

ANIMAL CELL; ARTICLE; BINDING SITE; CONTROLLED STUDY; GENE MUTATION; HEMOPHILIA A; HUMAN; HUMAN CELL; KIDNEY; MAMMAL CELL; MONKEY; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN DOMAIN; AMINO ACID SUBSTITUTION; ANIMAL; CELL LINE; CELL STRAIN COS1; GENETIC TRANSFECTION; GENETICS; METABOLISM; SITE DIRECTED MUTAGENESIS;

AMINO ACID SUBSTITUTION; ANIMALS; CELL LINE; COS CELLS; CYSTEINE ENDOPEPTIDASES; FACTOR VIII; HEMOPHILIA A; HUMANS; MUTAGENESIS, SITE-DIRECTED; NEOPLASM PROTEINS; PHOSPHATIDYLSERINES; PROTEIN BINDING; TRANSFECTION;

EID: 0034746156     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0037-1615697     Document Type: Article

References (28)

1. The life cycle of coagulation factor VIII in view of its structure and function
2. a from the isolated A1/A3-C1-C2 dimer and A2 subunit
3. Blood coagulation factors V and VIII: Structural and functional similarities and their relationship to hemorrhagic and thrombotic disorders
4. Molecular basis of factor VIII inhibition by human antibodies. Antibodies that bind to the factor VIII light chain prevent the interaction of VIII with phospholipid
5. 35S-labeled recombinant factor VIII to activated and unactivated human platelets
6. Deletion analysis of recombinant human factor V. Evidence for a phosphatidylserine binding site in the second C-type domain
7. Prothrombinase complex assembly on the platelet surface is mediated through the 74,000-dalton component of factor Va
8. Synthetic factor VIII peptides with amino acid sequences contained within the C2 domain of factor VIII inhibit factor VIII binding to phosphatidylserine
9. Some factor VIII inhibitor antibodies recognize a common epitope corresponding to C2 domain amino acids 2248 through 2312, which overlap a phospholipid-binding site
10. Crystal structures of the membrane-binding C2 domain of human coagulation factor V
11. Structure of the C2 domain of human factor VIII at 1.5 A resolution
12. Hemophilia A
13. The factor VIII structure and mutation resource site: HAMSTeRS version 4
14. Factor VIII C2 domain missense mutations exhibit defective trafficking of biologically functional proteins
15. The acidic region of the factor VIII light chain and the C2 domain together form the high affinity binding site for von Willebrand factor
16. Specificity of monoclonal antibodies to factor VIII:C
17. Effect of heterologous factor V heavy chain sequences on the secretion of recombinant human factor VIII
18. Expression and characterization of recombinant human factor V and a mutant lacking a major portion of the connecting region
19. A mechanism for inhibition of factor VIII binding to phospholipid by von Willebrand factor
20. A recombinant factor VIII A2 domain polypeptide quantitatively neutralizes human inhibitor antibodies that bind to A2
21. Reconstitution of human factor VIII from isolated subunits
22. Generation of active coagulation factor VIII from isolated subunits
23. Room temperature, microtray chromogenic assay of factor VIII:C
24. Structural basis for hemophilia A caused by mutations in the C domains of blood coagulation factor VIII
25. The canine factor VIII cDNA and 5′ flanking sequence
26. Identification of functionally important amino acid residues within the C2 domain of human factor V using alanine scanning mutagenesis
27. The phospholipid binding property of the C2 domain of human factor VIII
28. 2307→Gln mutation as a cause of haemophilia A