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Residual amounts of glycoprotein Ib concomitant with near-absence of glycoprotein IX in platelets of Bernard-Soulier patients

J Drouin, JL McGregor, S Parmentier, CA Izaguirre and KJ Clemetson

INSERM U. 63, Faculte de Medecine Alexis Carrel, Lyon, France.

A study of the Bernard-Soulier syndrome in two unrelated families using different polyclonal antibodies in a sensitive immunoblot assay showed residual amounts of platelet membrane glycoprotein (GP) lb in the eight homozygotes, as well as the near-absence of GPlb beta and GPIX. The eight heterozygotes studied showed a double band pattern for GPlb and about half the normal level of GPlb beta and GPIX. Therefore, we conclude that the Bernard-Soulier syndrome is heterogeneous and is probably not due to gene deletions.

Volume 72, Issue 3, pp. 1086-1088, 09/01/1988
Copyright © 1988 by The American Society of Hematology


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