Structural Biology
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Recent publications 

  • BALDUINI, C. L., SAVOIA, A. & SERI, M. 2013. Inherited Thrombocytopenias Frequently Diagnosed in Adults. Journal of Thrombosis and Haemostasis.
  • BERNDT, M. & ANDREWS, R. 2011. Bernard-Soulier Syndrome. Haematologica, 96, 355-359.
  • CHS, C. H. S. 2008. Bernard Soulier Syndrome - An Inherited Bleeding Disorder.
  • CLEMETSON, K. J. 2001. Platelet Glycoproteins and Their Role in Diseases. Transfusion Clinique et Biologique, 8, 155-162.
  • DU, X., BEUTLER, L., RUAN, C., CASTALDI, P. A. & BERNDT, M. C. 1987. Glycoprotein Ib and glycoprotein IX are fully complexed in the intact platelet membrane. Blood, 69, 1524-1527
  • HEMOPHILIA, W. F. O. 2012. About Bleeding Disorders: Bernard Soulier Syndrome [Online].Available: http://www.wfh.org/en/page.aspx?pid=657 [Accessed June 18th 2013.
  • LANZA, F. 2006. Bernard-Soulier Syndrome (Hemorrhagiparous thrombocytic dystrophy).
    Orphanet Journal of Rare Diseases, 1. 
  • LI, R. & EMSLEY, J. 2013. The Organizing Principle of the Platelet Glycoprotein Ib-IX-V
    Complex. Journal of Thrombosis and Haemostasis, 11, 605-614.
  • LUO, S.-Z., MO, X., AFSHAR-KHARGHAN, V., SRINIVASAN, S., LÓPEZ, J. A. & LI, R. 2007. Glycoprotein Ibα forms disulfide bonds with 2 glycoprotein Ibβ subunits in the resting platelet. Blood, 109, 603-609.
  • LÓPEZ, J. A., ANDREWS, R. K., AFSHAR-KHARGHAN, V. & BERNDT, M. C. 1998. Bernard-Soulier Syndrome. Blood, 91, 4397-4418.
  • MCEWAN, P. A., ANDREWS, R. K. & EMSLEY, J. 2009. Glycoprotein Ibalpha inhibitor
    complex structure reveals a combined steric and allosteric mechanism of von Willebrandfactor antagonism. Blood, 114, 4883-5.
  • MCEWAN, P. A., YANG, W., CARR, K. H., MO, X., ZHENG, X., LI, R. & EMSLEY, J. 2011. Quarternary organization of GPIb-IX complex and insights into Bernard-Soulier syndrome revealed by the structures of GPIb-beta and a GPIbbeta/GPIX chimera. Blood - Thrombosis and Haemostasis, 118, 5292-5301.
  • MHAWECH, P. & SALEEM, A. 2000. Inherited Giant Platelet Disorders: Classification and Literature Review. American Journal of Clinical Pathology, 113, 176-190.
  • MO, X., LIU, L., LOPEZ, J. A. & LI, R. 2012. Transmembrane domains are critical to the interaction between platelet glycoprotein V and glycoprotein Ib-IX complex. J Throm Haemost, 10, 1875-86.
  • NORIS, P., PERROTTA, S., BOTTEGA, R., PECCI, A. & AL, E. 2011. Clinical and laboratory features of 103 patients from 42 Italian families with inherited thrombocytopenia derived from the monoallelic Ala156Val mutation of GPIb-alpha (Bolzano mutation). Haematologica, 96
  • NURDEN, A. & NURDEN, P. 2011. Advances in Our Understanding of the Molecular Basis of Disorders of Platelet Function. Journal of Thrombosis and Haemostasis, 9, 76-91.
  • NURDEN, A. T. 1999. Inherited Abnormalities of Platelets. Thrombosis and Haemostasis, 82,
    468-480.
  • NURDEN, A. T. 2005. Qualitative Disorders of Platelets and Megakaryocytes. Journal of Thrombosis and Haemostasis, 3, 1773-1782.
  • OEHMCKE, S. & HERWALD, H. 2010. Contact system activation in severe infectious diseases.
    J. Molecular Medicine, 88, 121-126. 
  • PHAM, A. & WANG, J. 2007. Bernard Soulier Syndrome: An inherited Platelet Disorder. Arch Pathol Lab Med, 131, 1834-1836.
  • RAMASAMY, I. 2004. Inherited Bleeding Disorders: Disorders of platelet adhesion and aggregation. Critical Reviews in Oncology/Hematology, 49, 1-35.
  • SELIGSOHN, U. 2012. Treatment of Inherited Platelet Disorders. Haemophilia, 18, 161-165.
  • STRASSEL, C., DAVID, T., ECKLY, A., BAAS, M. J., MOOG, S., RAVANAT, C.,TRZECIAK, M. C., VINCIGUERRA, C., CAZENAVE, J. P., GACHET, C. & LANZA, F. 2006. Synthesis of GPIbb with novel transmembrane and cytoplasmic sequences in a Bernard-Soulier patient resulting in GPIb-defective signalling in CHO cells. Thrombosis and Haemostasis, 4, 217-28.
  • SUGI, T. & MAKINO, T. 2000. Plasma contact system, kallikrein-kinin system and antiphospholipid-protein antibodies in thrombosis. Journal of reproductive immunology, 47.
  • UFF, S., CLEMETSON, J. M., HARRISON, T., CLEMETSON, K. J., EMSLEY, J., 20, J. B. C.S. & 26., -. E. J. 2002. Crystal structure of the platelet glycoprotein Ib (alpha) N-terminal domain reveals an unmasking mechanism for receptor activation. J Biol Chem., 277, 35657-63.
  • WHITE, G. C. 2006. Congenital and Acquired Platelet Disorders: Current Dilemmas anTreatment Strategies. Seminars in Hematology, 43, S37-S41

Structural Biology

The University of Nottingham
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telephone: +44 (0)115 8467092
email:jonas.emsley@nottingham.ac.uk