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Carbohydrate deficiency of the factor VIII/von Willebrand factor Protein in von Willebrand's disease variants

H R Gralnick, B S Coller, Y Sultan

    Science (New York, N.Y.)
    |April 2, 1976
    PubMed

    Study of the normal human factor VIII/von Willebrand factor reveals a macromolecular glycoprotein composed of apparently identical subunits. This purified glycoprotein has procoagulant, antigen, and von Willebrand factor activities. In three patients with a variant of the von Willebrand's disease syndrome, their factor VIII/von Willebrand factor protein was present in normal amounts and had normal procoagulant and antigen activities; however, this protein was deficient in both carbohydrate and von Willebrand factor activity. The carbohydrate portion of the factor VIII/von Willebrand factor glycoprotein is of major importance in its interactions with platelets or the blood vessel wall, or both.

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