3-Dimensional structure of membrane-bound coagulation factor VIII: Modeling of the factor VIII heterodimer within a 3-dimensional density map derived by electron crystallography

Blood

Volumn 99, Issue 4, 2002, Pages 1215-1223

  Stoilova McPhie, Svetla ^{a,b,c,d,e,f,g}   Villoutreix, Bruno O ^a,b,c,d,e,f   Mertens, Koen ^a,b,c,d,e,f   Kemball Cook, Geoffrey ^a,b,c,d,e,f   Holzenburg, Andreas ^a,b,c,d,e,f  

^a BURNHAM INSTITUTE   (United States)

^b INSERM   (France)

^c CENTRAL LABORATORY   (Netherlands)

^d UTRECHT UNIVERSITY   (Netherlands)

^e HAMMERSMITH HOSPITAL   (United Kingdom)

^f TEXAS A AND M UNIVERSITY   (United States)

^g WADSWORTH CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BLOOD CLOTTING FACTOR 10; BLOOD CLOTTING FACTOR 8; BLOOD CLOTTING FACTOR 8A; BLOOD CLOTTING FACTOR 9A;

ARTICLE; CRYSTALLOGRAPHY; HUMAN; MOLECULAR MODEL; PHOSPHOLIPID MEMBRANE; PRIORITY JOURNAL; PROTEIN ASSEMBLY; PROTEIN DOMAIN; PROTEIN FOLDING; PROTEIN STRUCTURE; THREE DIMENSIONAL IMAGING;

EID: 0037082464     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood.V99.4.1215     Document Type: Article

References (73)

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10. Structure of the C2 domain of human FVIII at 1.5 Å resolution
11. Two-dimensional crystallization of proteins on planar lipid films and structure determination by electron crystallography
12. Structure of membrane-bound human factor Va
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14. A electron crystallography of human blood coagulation factor VIII bound to phospholipid monolayers
15. A molecular model for the triplicated A domains of human factor VIII based on the crystal structure of human ceruloplasmin
16. Factor VIII inhibitors in two families with mild haemophilia A: Structural analysis of the mutations
17. The factor VIII structure and mutation resource site: HAMSTeRS version 4
18. Small-volume extrusion apparatus for preparation of large, unilamellar vesicles
19. Three-dimensional structure of photosystem II
20. Three-dimensional structure determination by electron microscopy of two-dimensional crystals
21. Three-dimensional structure of membrane proteins determined by two-dimensional crystallization, electron cryomicroscopy and image analysis
22. Collaborative Computational Project No.4
23. Quantitative fitting of atomic models into observed densities derived by electron microscopy
24. Predicting antibody hypervariable loop conformation, I: Ensembles of random conformations for ring like structures
25. Prediction of protein secondary structure at better than 70% accuracy
26. Hemophilic factor VIII C1- and C2-domain missense mutations and their modeling to the 1.5-angstroms human C2-domain crystal structure
27. Molecular models for the two discoidin domains of human blood coagulation factor V
28. Binding of factor Villa and factor VIII to factor IXa on phospholipid vesicles
29. Identification of a binding site for blood coagulation factor IX on the light chain of human factor VIII
30. Activation of factor VIII by thrombin increases its affinity for binding to synthetic phospholipid membranes and platelets
31. Factor VIII-factor IX interactions: Molecular sites involved in enzyme-cofactor complex assembly
32. Protease and EGF1 domains of factor IXa play distinct roles in binding of factor Villa
33. Region 301-303 and 333-339 in the catalytic domain of blood coagulation factor IX are FVIII-interactive sites involved in stimulation of enzyme activity
34. Factor Villa A2 subunit residues 558-565 represent a factor IXa interactive site
35. The factor VIII peptide consisting of amino acids 698 to 712 enhances factor IXa cleavage of factor X
36. Synthetic peptides derived from residues 698 to 710 of factor VIII inhibit factor IXa activity
37. The sequence Gm1811-Lys1818 of human blood coagulation factor VIII comprises a binding site for activated factor IX
38. Factor IXa: Factor Villa interaction: Helix 330-338 of factor IXa interacts with residues 558-565 of the A2 subunit of factor Villa
39. The active site of factor IXa is located far above the membrane surface and its conformation is altered upon association with factor Villa
40. Interaction of the A1 subunit of factor Villa and the serine protease domain of factor X identified by zero-length cross-linking
41. Role of factor VIII C2 domain in factor VIII binding to factor Xa
42. Homology models of the C domains of blood coagulation factors V and VIII: A proposed membrane binding model for FV and FVIII C2 domains
43. Structural basis for hemophilia A caused by mutations in the C domains of blood coagulation factor VIII
44. Glycosylation pattern of a B-domain-deleted factor VIII molecule (r-VIII-SQ)
45. Structural characterization of site-specific N-glycosylation of recombinant human factor VIII by reversed-phase high-performance liquid chromatography-electrospray ionization mass spectrometry
46. Substitution of Arg527 and Arg531 in factor VIII associated with mild haemophilia A: Characterization in terms of subunit interaction and cofactor function
47. Human inhibitor antibodies for the factor VIII A2 domain disrupt the interaction between the subunit and factor IXa
48. Sites in the A2 subunit involved in the interfactor Villa interaction
49. Cleavage of FVIII heavy chain is required for the functional interaction of A2 subunit with FIXa
50. A novel cause of mild/moderate hemophilia A: Mutation scattered in the factor VIII C1 domain reduce the factor VIII binding to von Willebrand factor
51. Anti-factor VIII antibody inhibiting allogeneic but not autologous factor VIII in patient with mild hemophilia A
52. Some factor VIII (FVIII) inhibitors recognise a FVIII epitope(s) that is present only on FVIII-vWF complexes
53. A human antibody directed to the factor VIII C1 domain inhibits factor VIII cofactor activity and binding to von Willebrand factor
54. Relating structure to function: The role of the C2 domain in factor VIII
55. A role for the C2 domain of factor VIII binding to von Willebrand factor
56. A mechanism for inhibition of factor VIII binding to phospholipids by von Willebrand factor
57. The phospholipid binding properties of the C2 domain of human factor VIII
58. Synthetic factor VIII peptides with amino acid sequences contained within the C2 domain of FVIII inhibit FVIII binding to phosphatidyl serine
59. Membrane-binding peptide from the C2 domain of factor VIII forms an amphiphatic structure as determined by NMR spectroscopy
60. Use of surface plasmon resonance for studies of proteinprotein and protein-phospholipid membrane interactions: Application to the binding of factor VIII to von Willebrand factor and to phosphatidylserine containing membranes
61. Factor VIII C2 domain binds von Willebrand factor and phospholipid with high affinity and exhibits conformational flexibility
62. Residues Glu2181-Val2243 contain a major determinant of the inhibitory epitope in the C2 domain of human factor VIII
63. Four hydrophobic amino acids of the factor VIII C2 domain contribute to the membrane-binding motif
64. Antigenicity of putative phospholipid membrane-binding residues in factor VIII
65. Mutation in a potential phospholipid binding loop in the C2 domain of factor V affecting the assembly of the prothrombinase complex
66. Identification of functionally important amino acid residues within the C2-domain of human factor V using alanine-scanning mutagenesis
67. Sulfation of Tyr1680 of human blood coagulation factor VIII is essential for the interaction of factor VIII with von Willebrand factor
68. The affinity and stoichiometry of binding of human factor VIII to von Willebrand factor
69. The acidic region of the factor VIII light chain and the C2 domain together form the high affinity binding site for von Willebrand factor
70. Factor VIII's C1 domain enhances C2 binding of factors IX/IXa, X/Xa and von Willebrand factor (vWf)
71. Three-dimensional model of coagulation factor Va bound to activated protein C
72. Anticoagulant effect of a synthetic peptide containing residues Thr2253-Gln2270 within factor VIII C2 domain which selectively inhibits factor Xa-catalyzed factor VIII activation
73. Factor VIII C2 domain contains the thrombin-binding site responsible for thrombin-catalyzed cleavage at Arg 1689