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 Blood, 1 April 2007, Vol. 109, No. 7, pp. 2840-2846.
Prepublished online as a Blood First Edition Paper on November 21, 2006; DOI 10.1182/blood-2006-07-035105.

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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY

Effect of von Willebrand factor Y/C1584 on in vivo protein level and function and interaction with ABO blood group

James Anthony Davies1, Peter William Collins1, Lee Sarah Hathaway2, and Derrick John Bowen1

1 Department of Haematology, School of Medicine, Cardiff University, Cardiff; 2 University Hospital of Wales, Cardiff, United Kingdom.

Blood group O and the cysteine allele of the Y/C1584 change in von Willebrand factor (VWF) are enriched in type 1 VWD, but neither causes disease. We investigated the effect of C1584, alone and in combination with the ABO blood group, on the level and properties of plasma VWF. A cohort of 5052 blood donors was recruited: 50 donors were heterozygous for Y/C1584 and 5002 were homozygous for Y/Y1584. Mean VWF antigen (VWF:Ag) for heterozygotes (82 ± 35 IUdL–1) was significantly lower than for homozygotes (111 ± 37 IUdL–1) (P < .001). For each ABO blood group, VWF:Ag was decreased among Y/C1584 heterozygotes compared with Y/Y1584 homozygotes; a larger decrease was observed for group O. Among donors with VWF:Ag levels of 50 IUdL–1 or lower, Y/C1584 heterozygosity was markedly enriched (18%) compared with the entire cohort (1.5%). Blood group O was enriched to a lesser extent (2.4%), but Y/C1584 in conjunction with group O was strikingly enriched (34.8%). VWF collagen binding activity (VWF:CB) and ristocetin cofactor activity (VWF:RCo) were significantly lower for Y/C1584 heterozygotes than for Y/Y1584 homozygotes, and a qualitative difference in Y/C1584 plasma VWF multimer profile was observed compared with that for Y/Y1584 VWF. The data support a multifactorial basis for low VWF levels in some individuals.


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