My publications in biomedical science 1978-2020.
A. Book Chapters 1997 - present
Molecular Basis of Haemophilia A. G Kemball-Cook. In: Textbook of Haemophilia (Eds. Lee, Hoots and Berntorp). Blackwell, Oxford. Revised 3rd Edition published 2014.
Structure, Biology, and Genetics of Factor VIII. G Kemball-Cook, JH McVey and EGD Tuddenham. In: Hematology: Basic Principles and Practice, 5th Ed (Eds. Hoffman, Benz, Shattil, Furie, Silberstein, McGlave and Heslop). Churchill Livingstone, Oxford, 2008 Chapter 123.
Normal Haemostasis. G. Kemball-Cook and J.H. McVey. In: Postgraduate Haematology (Eds. Hoffbrand, Tuddenham and Catovsky), Blackwell, Oxford. 2004, pp783-807.
The molecular defect in haemophilia A. G Kemball-Cook and EGD Tuddenham. In: Haemophilia (Eds. Forbes, Aledort & Madhok), Chapman & Hall, London (1997) Chapter 3, pp21-33.
B. Original Articles in Peer-Reviewed Journals 1977 - present
77. The EAHAD blood coagulation factor VII variant database. Muriel Giansily‐Blaizot, Pavithra M. Rallapalli, Stephen J. Perkins, Geoffrey Kemball‐Cook, Daniel J. Hampshire, Keith Gomez, Christopher A. Ludlam and John H. McVey. Human Mutation (2020) 1-11 [Link to PDF]
76. The European Association for Haemophilia and Allied Disorders (EAHAD) Coagulation Factor Variant Databases: Important resources for haemostasis clinicians and researchers. John H. McVey, Pavithra M. Rallapalli, Geoffrey Kemball-Cook, Daniel J. Hampshire, Muriel Giansily-Blaizot, Keith Gomez, Stephen J. Perkins and Christopher A. Ludlam. Haemophilia (2020) 00:1–8. [Link to PDF]
75. An interactive mutation database for human coagulation factor IX provides novel insights into the phenotypes and genetics of haemophilia B. PM Rallapalli, G Kemball-Cook, EG Tuddenham, K Gomez and SJ Perkins. Journal of Thrombosis and Haemostasis (2013) 11, 1329-40. [PubMed Link]
74. A novel role for the Aurora B kinase in epigenetic marking of silent genes in differentiated post-mitotic cells. P Sabbattini, C Canzonetta, M Sjoberg, S Nikic, A Georgiou, G Kemball-Cook, H Auner and N Dillon. EMBO J. (2007) 26, 4657-69. [PubMed Link]
73. In silico prediction of FVIII epitopes recognised by natural autoantibodies in polyvalent immunoglobulin concentrates. M di Giambattista, T Branckaert, V Hougardy, G Kemball-Cook and R Laub. Molecular Immunology (2007) 44, 1903-1913. [PubMed Link]
72. Multiple vitamin K-dependent coagulation zymogens promote adenovirus-mediated gene delivery to hepatocytes in vitro and in vivo. AL Parker, SN Waddington, CG Nicol, DM Shayakhmetov, SMK Buckley, L Denby, G Kemball-Cook, S Ni, A Lieber, JH McVey, SA Nicklin and AH Baker. Blood (2006) 108, 2554-2561. [PubMed Link]
71. Post-injury vascular intimal hyperplasia in mice is completely inhibited by CD34+ bone marrow-derived progenitor cells expressing membrane-tethered anticoagulant fusion proteins. D. Chen, M. Weber, P.G. Shiels, R. Dong, Z. Webster, J. H. McVey, G. Kemball-Cook, E. G. D. Tuddenham, R. I. Lechler and A. Dorling. Journal of Thrombosis and Haemostasis (2006) 4, 2191-2198. [PubMed Link]
70. The spectrum of mutations and molecular pathogenesis of haemophilia A in 181 Portuguese patients. D David, C Ventura, I Moreira, MJ Diniz, M Antunes, A Tavares, F Araujo, S Morais, M Campos, J Lavinha and G Kemball-Cook. Haematologica (2006) 91, 840-843. [PubMed Link]
69. Self complementary adeno-associated viral vectors containing a novel liver-specific human factor IX expression cassette enable highly efficient transduction of murine and nonhuman primate liver. Amit C. Nathwani, John T. Gray, Catherine Y.C. Ng, Junfang Zhou, Yunyu Spence, Simon Waddington, Edward GD Tuddenham, Geoffrey Kemball-Cook, Jenny McIntosh, Mariette Boon- Spijker, Koen Mertens and Andrew M. Davidoff Blood (2006) 107, 2653-2661. [PubMed Link]
68. Permanent partial phenotypic correction and tolerance in a mouse model of haemophilia B by stem cell gene delivery of human factor IX. B Bigger, E Sipiati, A Mistry, S Waddington, M Nivsarkar, L Jacobs, R Perrett, M Holder, C Ridler, G Kemball-Cook, R Ali, S Forbes, C Coutelle, N Wright, M Alison, A Thrasher, D Bonnet, M Themis. Gene Therapy (2006) 13, 117-126. [PubMed Link]
67. Characterisation of blood coagulation factor XI T475I. JH McVey, K Lal, Y Imanaka, G Kemball- Cook, PHB Bolton-Maggs and EG Tuddenham. Thrombosis and Haemostasis (2005) 93, 1082-8. [PubMed Link]
66. Generation of a polyclonal rabbit anti-mouse tissue factor antibody by nucleic acid immunization. AD Mumford, D Chen, A Dorling, G Kemball-Cook and JH McVey Thrombosis and Haemostasis,(2005) 93, 160-164. [PubMed Link]
65. Permanent phenotypic correction of haemophilia B in immunocompetent mice by prenatal gene therapy. SW Waddington, M Nivsarkar, A Mistry, SMK Buckley, F Al-Allaf, B Bigger, M Holder, G Kemball-Cook, KL Mosley, M Brittan, L Gregory, HT Cook, A Thrasher, EGD Tuddenham, I Verma, M Themis and C Coutelle. Blood (2004) 104, 2714-2721. [PubMed Link]
64. Complete inhibition of acute humoral rejection by regulated expression of endothelial cell-tethered anticoagulants on transplanted xenografts. D Chen, M Weber, J McVey, G Kemball-Cook, E Tuddenham, R Lechler, A Dorling. J Am Transp (2004) 4, 1958-63. [PubMed Link]
63. Inhibition of intravascular thrombosis in murine endotoxaemia by targeted expression of hirudin and tissue factor pathway inhibitor analogues to activated endothelium. D Chen, K Giannopoulos, P Shiels, Z Webster, J McVey, G Kemball-Cook, E Tuddenham, M Moore, R Lechler, A Dorling. Blood, (2004) 104, 1344-1349. [PubMed Link]
62. Two novel mutations in severe factor VII deficiency. Keith Gomez, Michael A. Laffan, Geoffrey Kemball-Cook, John Pasi, Mark Layton, Jack D. Singer, Edward G.D. Tuddenham and John H. McVey. British Journal of Haematology, (2004) 126, 105-110. [PubMed Link]
61. In utero transfer of human factor IX to fetal mice can induce postnatal tolerance of the exogenous clotting factor. SW Waddington, SMK Buckley, M Nivsarkar, S Jezzard, H Schneider, T Dahse, G Kemball-Cook, M Miah, N Tucker, MJ Dallman, M Themis and C Coutelle. Blood (2003) 101, 1359-1366. [PubMed Link]
60. Hemophilia A Mutations Within the Factor VIII A2-A3 Subunit Interface Destabilize Factor VIIIa and cause One-stage/Two-stage Activity Discrepancy. WH Hakeos, H Miao, N Sirachainan, G Kemball-Cook, EL Saenko, RJ Kaufman and SW Pipe. Thrombosis and Haemostasis (2002) 88, 781-7. [PubMed Link]
59. Factor VIII – Novel Insights into Form and Function. EL Saenko, NM Ananyeva, EGD Tuddenham and G Kemball-Cook. British Journal of Haematology (2002) 119, 323-331. [Review] [PubMed Link]
58. Use of a non-depleting anti-CD4 antibody to modulate the immune response to coagulation factors VIII and IX. N Salooja, G Kemball-Cook, EGD Tuddenham and Julian Dyson. British Journal of Haematology (2002) 118, 839-42. [PubMed Link]
57. A Tyr346>Cys substitution in the inter-domain acidic region a1 of factor VIII in an individual with factor VIII:C assay discrepancy. AD Mumford, M Laffan, J O’Donnell, JH McVey, DJD Johnson, RA Manning and G Kemball-Cook. British Journal of Haematology (2002) 118, 589-594. [PubMed Link]
56. 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. S Stoilova-McPhie, B Villoutreix, K Mertens, G Kemball-Cook and A Holzenburg. Blood (2002) 99, 1215-1223. [PubMed Link]
55. Human thrombin and factor Xa mediate porcine endothelial cell activation; modulation by expression of TFPI-CD4 and hirudin-CD4 fusion proteins. D Chen, K Riesbeck, JH McVey, G Kemball-Cook, EGD Tuddenham, RI Lechler and A Dorling. Xenotransplantation (2001) 8, 258-265. [PubMed Link]
54. Stable recombinant expression and characterisation of the two haemophilic FVIII variants C329S (CRM-) and G1948D (CRMr). D David, EL Saenko, IMA Santos, DJD Johnson, EGD Tuddenham, JH McVey and G Kemball-Cook. British Journal of Haematology (2001) 113, 604-615. [PubMed Link]
53. Haemophilia A mutations associated with one-stage/two-stage activity discrepancy disrupt protein-protein interactions between the A1 and A2 subunits of factor VIII. SW Pipe, AN Eickhorst, EL Saenko, G Kemball-Cook and RJ Kaufman. Blood (2001) 97, 685-691. [PubMed Link]
52. Factor VII deficiency and the FVII mutation database. JH McVey, E Boswell, AD Mumford, G Kemball-Cook and EGD Tuddenham. Human Mutation (2001) 17, 3-17. [PubMed Link]
51. Contact activation in shock caused by invasive Group A Streptococcus pyogenes. S Sriskandan, G Kemball-Cook, D Moyes, J Canvin, EGD Tuddenham and J Cohen. Critical Care Medicine (2000) 28, 3684-91. [PubMed Link]
50. Molecular analysis of the genotype-phenotype relationship in factor VII deficiency. DS Millar, G Kemball-Cook, JH McVey, EGD Tuddenham, AD Mumford, GB Attock, JC Reverter, N Lanir, LA Parapia, J Reynaud, E Meili, A von Felton, U Martinowitz, DR Prangnell, M Krawczak and DN Cooper. Human Genetics (2000) 107, 327-342. [PubMed Link]
49. Assay discrepancy in mild haemophilia A due to a factor VIII missense mutation (Asn694Ile) in a large Danish family. R Schwaab, J Oldenburg, G Kemball-Cook, T Albert, C Juhler, P Hanfland and J Ingerslev. British Journal of Haematology (2000) 109, 523-528. [PubMed Link]
48. Electron crystallography of human blood coagulation factor VIII bound to phospholipid monolayers. SS Stoylova, PJ Lenting, G Kemball-Cook and A Holzenburg. Journal of Biological Chemistry (1999) 274, 36573-36578. [PubMed Link]
47. Crystal structure of active site-inhibited human coagulation factor VIIa (des-Gla). G Kemball-Cook, DJD Johnson, EGD Tuddenham and K Harlos. Journal of Structural Biology (1999) 127, 213-223. [PubMed Link]
46. Regulated inhibition of coagulation by porcine endothelial cells expressing P-selectin-tagged hirudin and tissue factor pathway inhibitor fusion proteins. D Chen, JH McVey, G Kemball-Cook, EGD Tuddenham, RI Lechler and A Dorling. Transplantation (1999) 68, 832-839. [PubMed Link]
45. Diagnostic importance of the two-stage factor VIII:C assay demonstrated by a case of mild haemophilia associated with His1954 ÷Leu substitution in the factor VIII A3 domain. DM Keeling, K Sukhu, G Kemball-Cook, N Waseem, R Bagnall and JV Lloyd. British Journal of Haematology (1999) 105, 1123-1126. [PubMed Link]
44. Inhibition of tissue factor-dependent and -independent coagulation by cell surface expression of novel anticoagulant fusion proteins. D Chen, K Riesbeck, G Kemball-Cook, JH McVey, EGD Tuddenham, RI Lechler and A Dorling. Transplantation (1999) 67, 467-474. [PubMed Link]
43. Crystallization and preliminary X-ray analysis of active site-inhibited human coagulation factor VIIa (des-Gla). DJD Johnson, PG Nugent, EGD Tuddenham, K Harlos and G Kemball-Cook. Journal of Structural Biology (1999) 125, 90-93. [PubMed Link]
42. Expression of hirudin fusion proteins in mammalian cells: a strategy for prevention of intravascular thrombosis. K Riesbeck, D Chen, G Kemball-Cook, JH McVey, AJT George, EGD Tuddenham, A Dorling and RI Lechler. Circulation (1998) 98, 2744-2752. [PubMed Link]
41. The solution structure of human coagulation factor VIIa in its complex with tissue factor is similar to free factor VIIa: a study of a heterodimeric receptor/ligand complex by X-ray and neutron scattering and computational modelling. AW Ashton, MK Boehm, DJD Johnson, G Kemball-Cook and SJ Perkins. Biochemistry (1998) 37, 8208-8217. [PubMed Link]
40. Structural determination of lipid-bound human blood coagulation factor IX. S Stoylova, E Gray, TW Barrowcliffe, G Kemball-Cook and A Holzenburg. Biochimica et Biophysica Acta (1998) 1383, 175-178. [PubMed Link]
39. Coagulation Factor VII Gln100>Arg: amino acid substitution at the epidermal growth factor 2 protease domain interface results in severely reduced tissue factor binding and procoagulant function.G Kemball Cook, DJD Johnson, O Takamiya, DW Banner, JH McVey, and EGD Tuddenham. Journal of Biological Chemistry (1998) 273, 8516-8521. [PubMed Link]
38. The molecular basis for cross-reacting material-positive hemophilia A due to missense mutation within the A2-domain of factor VIII. K Amano, R Sarkar, S Pemberton, G Kemball-Cook, HH Kazazian and RJ Kaufman. Blood (1998) 91, 538-548. [PubMed Link]
37. The factor VIII Structure and Mutation Resource Site: HAMSTeRS Version 4. G Kemball-Cook, EGD Tuddenham and AI Wacey. Nucleic Acids Research (1998) 26, 216-219. [PubMed Link]
36. Human tissue factor pathway inhibitor fused to CD4 binds both FXa and TF/FVIIa at the cell surface. K Riesbeck, A Dorling, G Kemball-Cook, JH McVey, M Jones, EGD Tuddenham and RI Lechler: Thrombosis and Haemostasis (1997) 78, 1488-1494. [PubMed Link]
35. Homology modelling of the catalytic domain of early mammalian protein C: evolution of structural features. AI Wacey, M Krawczak, G Kemball-Cook and DN Cooper. Human Genetics (1997), 101, 37-42. [PubMed Link]
34. High prevalence of elevated factor VIII levels in patients referred for thrombophilia screening: role of increased synthesis and relationship to the acute phase reaction. J O’Donnell, EGD Tuddenham, R Manning, G Kemball-Cook, D Johnson and M Laffan. Thrombosis and Haemostasis (1997), 77, 825-828. [PubMed Link]
33. A molecular model for the triplicated A domains of human factor VIII based on the crystal structure of human caeruloplasmin. S Pemberton, P Lindley, V Zaitsev, G Card, EGD Tuddenham and G Kemball-Cook. Blood (1997), 89, 2413-2421. [PubMed Link]
32. The factor VIII mutation database on the World Wide Web: the haemophilia A mutation, search, test and resource site. HAMSTeRS v3.0. G Kemball-Cook and EGD Tuddenham. Nucleic Acids Research (1997), 25, 128-132. [PubMed Link]
31 Pharmacokinetic in vivo comparisons using 1-stage and chromogenic substrate assays with two formulations of Hemofil-M. CA Lee, TW Barrowcliffe, G Bray, E Gomperts, AR Hubbard, G Kemball-Cook, P Lilley, D Owens, L von Tilberg and J Pasi. Thrombosis and Haemostasis (1996), 76, 950-6. [PubMed Link]
30. In vivo studies of activated porcine factor VIII. JD Littlewood, SA Bevan, G Kemball-Cook and TW Barrowcliffe. Thrombosis and Haemostasis (1996), 76, 743-8. [PubMed Link]
29. The haemophilia A mutation search test and resource site, home page of the factor VIII mutation database: HAMSTeRS. AI Wacey, G Kemball-Cook, HH Kazazian, SE Antonarakis, R Schwaab, P Lindley and EGD Tuddenham. Nucleic Acids Research (1996), 24, 100-102. [PubMed Link]
28. Factor VIIa and the extracellular domains of human tissue factor form a compact complex: a study by X-rays and neutron solution scattering. AW Ashton, G Kemball-Cook, DJD Johnson, DMA Martin, DP O’Brien, EGD Tuddenham and SJ Perkins. FEBS Letters (1995), 374, 141-146. [PubMed Link]
27. Measurement of activated factor IX in factor IX concentrates: correlation with in vivo thrombogenicity. E Gray, JE Tubbs, SJ Thomas, AD Oates, M Boisclair, G Kemball-Cook and TW Barrowcliffe. Thrombosis and Haemostasis (1995), 73, 675-679. [PubMed Link]
26. Surface plasmon resonance studies of the interaction between factor VII and tissue factor. Demonstration of defective tissue factor binding in a variant FVII molecule (FVII-R79Q). DP O'Brien, G Kemball-Cook, AM Hutchinson, DMA Martin, DJ Johnson, O Takamiya, EGD Tuddenham and JH McVey. Biochemistry (1994), 33, 14162-14169. [PubMed Link]
25. High level expression of human blood coagulation factors VII and XI: use of a new mammalian expression vector. G Kemball-Cook, I Garner, Y Imanaka, T Nishimura, DP O'Brien, EGD Tuddenham and JH McVey. Gene (1994), 139, 275-279. [PubMed Link]
24. Detection of missense mutations by single-strand conformational polymorphism (SSCP) analysis in five dysfunctional variants of coagulation factor VII. O Takamiya, G Kemball-Cook, DMA Martin, DN Cooper, A von Felten, E Meili, I Hann, DR Prangnell, H Lumley, EGD Tuddenham and JH McVey. Human Molecular Genetics (1993), 2, 1355-1359. [PubMed Link]
23. Inhibitor development and activated factor VIII in concentrates. TW Barrowcliffe, G Kemball-Cook and JE Tubbs. Thrombosis and Haemostasis (1993), 70, 1065-1066. [PubMed Link]
22. The behaviour of different factor VIII concentrates in a chromogenic factor X-activating system. G Kemball-Cook, JE Tubbs, NJ Dawson and TW Barrowcliffe. British Journal of Haematology (1993) 84, 273-278. [PubMed Link]
21. Interaction of factor VIII and phospholipids: role of composition and negative charge. G Kemball- Cook and TW Barrowcliffe. Thrombosis Research (1992), 67, 57-71. [PubMed Link]
20. Proteolysis of factor VIII heavy chain polypeptides in plasma and concentrates. G Kemball-Cook, SJ Edwards and TW Barrowcliffe (1991). British Journal of Haematology, 78, 222-228. [PubMed Link]
19. Variable inactivation of human factor VIII from different sources by human factor VIII inhibitors. JD Littlewood, SA Bevan, G Kemball-Cook, RJ Evans and TW Barrowcliffe. British Journal of Haematology (1991), 77, 535-538. [PubMed Link]
18. Factor VIII heavy chain polypeptides in plasma and concentrates. G Kemball-Cook, SA Bevan and TW Barrowcliffe. British Journal of Haematology (1990), 76, 80-87. [PubMed Link]
17. Potency of high purity factor VIII concentrates. TW Barrowcliffe, J Watton, A Harman, JE Tubbs and G Kemball-Cook. Lancet (1990) July 14, 124. [PubMed Link]
16. Assay discrepancies with highly purified factor VIII concentrates. NJ Dawson, G Kemball-Cook and TW Barrowcliffe. Haemostasis (1989), 19, 131-137. [PubMed Link]
15. Factor VIII procoagulant protein interacts with phospholipid vesicles via its 80kDa light chain. G Kemball-Cook, SJ Edwards, K Sewerin, L-O Andersson and TW Barrowcliffe. Thrombosis and Haemostasis (1988), 60, 442-446. [PubMed Link]
14. Interaction of factor VIII clotting protein with phospholipids. G Kemball-Cook. Ph.D thesis, CNAA (1987).
13. The effect of Ca2+, phospholipid and factor V on the anti-(factor Xa) activity of heparin and its high-affinity oligosaccharides. TW Barrowcliffe, SJ Havercroft, and G Kemball-Cook. Biochemical Journal (1987), 243, 31-37. [PubMed Link]
12. Assay of factor VIII concentrates: comparison of the chromogenic and two-stage assays. AR Hubbard, AD Curtis, TW Barrowcliffe, SJ Edwards, CA Jennings and G Kemball-Cook. Thrombosis Research (1986), 44, 887-891. [PubMed Link]
11. Factor VIII degradation products in heated concentrates. TW Barrowcliffe, SJ Edwards, G Kemball-Cook and DP Thomas. Lancet (June 21 1986), 1448-1449. [PubMed Link]
10. Factor VIII concentrates contain factor VIII procoagulant antigen bound to phospholipid. G Kemball-Cook and TW Barrowcliffe. British Journal of Haematology (1986), 63, 425-434. [PubMed Link]
9. Effect of phospholipid on factor VIII inactivation. TW Barrowcliffe, G Kemball-Cook and E Gray. In: Factor VIII Inhibitors (Alan R Liss, New York) (1984), 251-263. [PubMed Link]
8. Overcoming factor VIII inhibitors- a possible new approach. TW Barrowcliffe, E Gray and G Kemball-Cook. Scandinavian Journal of Hematology (1984) 33, Suppl 40, 207-212. [PubMed Link]
7. Fractionation of human antibody to FVIII:C: an IRMA for phospholipid binding sites on FVIIIC:Ag. G Kemball-Cook and TW Barrowcliffe. British Journal of Haematology (1984), 57, 633-636. [PubMed Link]
6. Binding to phospholipid protects factor VIII from inactivation by human antibodies. TW Barrowcliffe, G Kemball-Cook and E Gray. Journal of Laboratory and Clinical Medicine (1983), 101, 34-43. [PubMed Link]
5. Aluminium hydroxide absorption of factor VIII procoagulant antigen from plasma and factor VIII concentrates. G Kemball-Cook, RA Furlong, IR Peake and TW Barrowcliffe. Thrombosis Research (1981), 23, 193-196. [PubMed Link]
4. Factor VIII-related activities in therapeutic concentrates. TW Barrowcliffe, G Kemball-Cook, G Morris, JC Holt, RA Furlong and IR Peake. Journal of Laboratory and Clinical Medicine (1981), 97, 429-438. [PubMed Link]
3. Factor VIII inhibitor bypassing activity: a suggested mechanism of action. TW Barrowcliffe, G Kemball-Cook and E Gray. Thrombosis Research (1981), 21, 181-186. [PubMed Link]
2. Adsorption of factor VIII by Al(OH)3. Seghatchian MJ, G Kemball-Cook and TW Barrowcliffe. Haemostasis (1979), 8, 106-116. [PubMed Link]
1. Anticoagulant activities of high and low molecular weight heparin fractions. TW Barrowcliffe, EA Johnson, CA Eggleton, G Kemball-Cook and DP Thomas. British Journal of Haematology (1979), 41, 573-583. [PubMed Link]