Molecular defects of platelets in Bernard-Soulier syndrome.

  title={Molecular defects of platelets in Bernard-Soulier syndrome.},
  author={Paquita Nurden and Dominique Didry and Jean Philippe Rosa},
  journal={Blood cells},
  volume={9 2},
In 1969, it was shown that platelets of patients with Bernard-Soulier (B-S) syndrome exhibited an altered electrophoretic mobility arising from a surface membrane sialic acid deficiency. Subsequent studies showed that a major membrane glycoprotein, GP Ib, was either deleted, reduced in concentration, or structurally modified in B-S platelets. Controversy persists, however, as to the specificity of the defect in platelets of different patients, especially with regard to studies performed on… CONTINUE READING


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