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Blood, 15 September 2005, Vol. 106, No. 6, pp. 1988-1991. Prepublished online as a Blood First Edition Paper on May 10, 2005; DOI 10.1182/blood-2005-02-0792. This Article Full Text (PDF) All Versions of this Article: 2005-02-0792v1 106/6/1988    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by O'Donnell, J. S Articles by Laffan, M. A Search for Related Content PubMed PubMed Citation Articles by O'Donnell, J. S Articles by Laffan, M. A Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted February 25, 2005 Accepted May 4, 2005 Bombay phenotype is associated with reduced plasma VWF levels and an increased susceptibility to ADAMTS13 proteolysis James S O'Donnell*, Thomas A McKinnon, James T Crawley, David A Lane, and Michael A Laffan Haematology Dept., Imperial College London, Hammersmith Hospital Campus, London, United Kingdom * Corresponding author; email: j.odonnell{at}imperial.ac.uk. ABO blood group is an important determinant of plasma VWF:Ag levels, with lower levels in group O. Previous reports have suggested that ABO(H) sugars affect the susceptibility of VWF to ADAMTS13 cleavage. To further test this hypothesis, we collected plasma from individuals with the rare Bombay blood group. VWF:Ag levels were significantly lower in Bombay patients (median 0.69 IU/ml) than in groups AB, A or B (P< 0.05) and lower than in group O individuals (median 0.82 IU/ml). Susceptibility of purified VWF fractions to recombinant ADAMTS13 cleavage, assessed using VWF:CB, was increased in Bombays compared to either group O or AB. Increasing urea concentration (0.5 to 2M) increased the cleavage rate for each blood group, but eliminated the differences between groups. We conclude that reduction in the number of terminal sugars on N-linked glycan increases susceptibility of globular VWF to ADAMTS13 proteolysis, and is associated with reduced plasma VWF:Ag and VWF:CB levels. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: R. de Groot, A. Bardhan, N. Ramroop, D. A. Lane, and J. T. B. Crawley Essential role of the disintegrin-like domain in ADAMTS13 function Blood, May 28, 2009; 113(22): 5609 - 5616. [Abstract] [Full Text] [PDF] L. Gallinaro, M. G. Cattini, M. Sztukowska, R. Padrini, F. Sartorello, E. Pontara, A. Bertomoro, V. Daidone, A. Pagnan, and A. Casonato A shorter von Willebrand factor survival in O blood group subjects explains how ABO determinants influence plasma von Willebrand factor Blood, April 1, 2008; 111(7): 3540 - 3545. [Abstract] [Full Text] [PDF] T. A. J. McKinnon, A. C. K. Chion, A. J. Millington, D. A. Lane, and M. A. Laffan N-linked glycosylation of VWF modulates its interaction with ADAMTS13 Blood, March 15, 2008; 111(6): 3042 - 3049. [Abstract] [Full Text] [PDF] M. Nosaka, Y. Ishida, A. Tanaka, T. Hayashi, T. Miyashita, C. Kaminaka, W. Eisenmenger, F. Furukawa, and A. Kimura Aberrant Expression of Histo-blood Group A Type 3 Antigens in Vascular Endothelial Cells in Inflammatory Sites J. Histochem. Cytochem., March 1, 2008; 56(3): 223 - 231. [Abstract] [Full Text] [PDF] J. A. Davies, P. W. Collins, L. S. Hathaway, and D. J. Bowen Effect of von Willebrand factor Y/C1584 on in vivo protein level and function and interaction with ABO blood group Blood, April 1, 2007; 109(7): 2840 - 2846. [Abstract] [Full Text] [PDF] J. A. Davies and D. J. Bowen An association between the L1565 variant of von Willebrand factor and susceptibility to proteolysis by ADAMTS13 Haematologica, February 1, 2007; 92(2): 240 - 243. [Abstract] [Full Text] [PDF]

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